Usmle World Heme/onc

Front 1

What is polycythemia vera, how does it present, what do labs show, what is mutation?

Back 1

Myeloproliferative disorder. Can by primary or secondary. Primary is a clonal BM disorder resulting in EPO-independent overproduciton of RBC -> serum EPO levels are LOW. JAK2 V617F motation in >95%. Symptoms: headache, dizziness, visual disturbances are frequnetly related to hyperviscosity. PRURITIS (esp after warm shower) due to release of histmaine mast cell degranulation) and prostaglandins (ex: stimuli from RBC) present in 40%. High histamine release is also responsible for GI symptoms (ex: peptic ulceration). HTN frequently occurs due to inc BV and is reversible by lowering Hct. PE: facial plethora, splenomegaly, hepatomegaly. Labs: INC HB/HCT, INC PLATELETS, INC WBC. Pts are at risk for both thrombosis (bc of high platelet count) and bleeding (due to impaired platelet function). Usually have norlam O2 sat levels and BM is hypercellular

Front 2

What happens to EPO and serium iron levels in polycythemia vera?

Back 2

EPO and iron levels are low (iron low due to inc hematopiesis and iron utilization in Hb synthesis). EPO low (in primary) bc EPO-independent

Front 3

When do you see BCR-ABL fusion protein?

Back 3

CML

Front 4

What is the workup algorithm for DVT?

Back 4

If pretest probability of DVT using Wells is low, do D-dimer (if elevated, do compression U/S). If high, do compression U/S (if positive, treat with heparin and warfarin; if neg, unlikely to have DVT - if still suspicious, do contrast venography).

Front 5

How does DVT present? What is ddx

Back 5

pain, swelling, discoloration of leg. Ddx: Baker's cyst, venous insufficiency, post-thrombotic syndrome, cellulitis

Front 6

What is the preferred initial test in pts with high pretest probability for DVT?

Back 6

Compression US. lack of compressibility of vein with US probe is highly sensitive and specific for dx of proximal DVT. If see it, should start anticoaguliation; unlike PE, no need to start immediate anticoag before testing pts in high DVT probability - can get US very quickly

Front 7

How do you treat DVT?

Back 7

Anticoatulation - heparin then warfarin (heparin first bc of warfarin's initial prothrombotic potential and delayed action)

Front 8

What are the lab values in tumor lysis syndrome? When does it occur?

Back 8

HyperP, hypoCa, hyperK, hyperuricemia. Tumors with high cell turnovers (poorly differentiated lymphomas and leukemias - Burkitts, ALL, less often AML) are frequent culprits. Both K and P are intracellular ions, so they are released when cells break down. Released P binds Ca -> less Ca. High uric acid due to degradation of cellular proteins.

Front 9

WHat can you use to reduce risk of urate nephropathy in tumor lysis syndrome?

Back 9

Allopurinol (but tumor lysis still possible)

Front 10

Why is it important to promptly identify and treat electrolyte abnormalities of tumor lysis syndrome?

Back 10

Bc they can lead to fatal arrhythmias, acure renal failure, and even sudden death

Front 11

What should you suspect in unexplained hemolytic anemia and thrombocytopenia in pt with renal failure and neurologic symptoms? What will see on peripheral smear?

Back 11

TTP-HUS. Dx: fragmented cells in peripheral smear suggests microangiopathic hemolytic anemia (MAHA) - a characteristic finding in TTP-HUS

Front 12

What causes idiopathic TTP-HUS?

Back 12

Deficinecy of or autoAb against a specific von Willebrand factor-cleaving protease (ADAMTS-13) -> casues accumulation of large vWF multimers and platelet aggregation.

Front 13

How can you treat TTP-HUS and what happens if you don't?

Back 13

80% fatal if no approrpiate therapy promptly. Plasmaphersis (plasma exchange) is treatment of choice and should be started as soon as possible. It removes the offending AutoAb and repletes the deficient enzyme. Platelet transfusion contraindicated bc can worsen the renal failure and neurologic symptoms due to continued consumption of platelets by microthrombi.

Front 14

When are PRBC indicated?

Back 14

If healthy, Hb <7-8. For CAD and CHF, Hv <10

Front 15

When is cryoprecipitate infusion used?

Back 15

Contains fibrinogen and is used to treat low fibrinogen states

Front 16

When is serum Ag CA125 useful?

Back 16

Can help dx ovarian cancer in pts with symptoms or as a screening tool in pts with inc risk secondary to hereditary factors (ex: BRCA+). Not recommended for pts at average risk

Front 17

What is the cancer with the highest GYN mortality rate in US? How screen?

Back 17

Ovarian cancer - but, no screening recommended for pts with average risk. Pelvic (transvaginal) US and serum CA125 Ag can help dx if suggestive symptoms or PE findings or if BRCA+

Front 18

When is CA125 elevated?

Back 18

50% of early ovarian cancers and 80% of advanced cancers -> not specific enough for a screening test (won't dec mortality)

Front 19

What is the management after dx of solid testicular mass has been made (and what does that mean)?

Back 19

Solid testicualr mass = painless hard mass in testicle + suggestive US. Initial management is removal of testis and its associated cord, orchiectomy (called a high inguinal orchiectomy). Depending on cell type, additional surgery, radiation therapy, or possibly chemo may be indicated. Dont' want to bx of of high risk of spillage of cancer cells, which can potentially spread through lymphatics and BC

Front 20

What is the single most prognostic consideration in treatment of pts with breast cancer?

Back 20

TMN staging (tumor burden) - worst prognosis is stage IV

Front 21

What does overexpression of Her-2/neu oncogene mean for prognosis of breast cancer

Back 21

worse prognosis

Front 22

What does ER+ and PR+ in breast cancer mean for prognosis?

Back 22

better prognosis

Front 23

What is the most common cause of B12 deficiency and what cancer is it assoc with?

Back 23

Pernicious anemia. At least double risk of gastric cancer.

Front 24

What are s/s of B12 deficiency?

Back 24

Macrocytic anemia, glossitis, neurologic changes including peripheral neuropathy.

Front 25

How does pernicious anemia lead to B12 deficiency?

Back 25

2 ways - 1st, anti-intrinsic factor Ab decrease the amount of functional intrinsic factor available to facilitate B12 absorption. 2nd - pts develop a chronic atrophic gastritis with dec production of intrinsic factor by gastric pariental cells. This atrophic gastritis increases the risk of intestinal-type gastric cancer and gastric carcinoid tumors by 2-3 times over the general population - thus, pts with pernicious anemia need to be monitored for development of gastric caner (periodic stool testing for presence of blood)

Front 26

What are post-splenectomy pts at risk for and why?

Back 26

Inc risk for sepsis from encapsulated organisms due to impaired Ab-mediated opsonization in phagocytosis. Normally, blood-borne Ag enter spleen via the splenic artery and are phagocytosed by dendritic cells in the white pulp - those dentritic cells then present Ag in association with MHC II to Th cells, activating them. Activated Th cells then migrate to the marginal zone of the spleen where they come into contact with B-cells in primary follicles. B-cell activation causes secondary follicles and plasma cell-rich germinal centers form. Ab produced by germinal center plasma cells enter the systemic circulation, bind their specific Ag, and facilitate phagocytosis of pathogenic organisms by opsonization. Asplenic pts are less able to mount this sort of Ag-specific Ab response and are therefore at high risk of overwhelming infectoin by encapsulated organisms like S. pneumoniae, N. meningitidis, and H. flu

Front 27

What disease has defective intracellular killing

Back 27

Chronic granulomatous disease (X-linked recessive (or autosomal recessive) defect on NADPH oxidase)

Front 28

When are the number of circulating L's selectively decreased?

Back 28

SCID and HIV

Front 29

What is the most common cause of SCID?

Back 29

Autosomal recessive defect in adenosine deaminase

Front 30

When is chemotaxis impaired (what disease)?

Back 30

Leukocyte adhesion defect (AR defect in integrin B2)

Front 31

What conditions are assoc with thymic aplasia and what is the cause?

Back 31

DiGeorge syndrome (result of deletion in chr22) - cause a defective cell-mediate immunity

Front 32

What happens to pts with underlying congenital protein C deficiency that are given warfarin?

Back 32

Warfarin inhibits protein C and S first, resulting in a hypercoagulable state and placing pt at risk for thormbus formation and skin necrosis, which is inc in pts with protein C deficinecy

Front 33

What does antithrombin III deficiency predispose to?

Back 33

Thrombus formation. It is a vit K-independent inhibitor of the clotting cascade

Front 34

What does Factor VII deficiency cause?

Back 34

Bleeding diathesis characerized by bruising and hemorrhage.

Front 35

What happens to pts who have excessive platelet aggregation and how does this occur?

Back 35

Known to occur in a small % of pts started on heparin - this paradoxical pro-thormbotic state (known as HITT - heparin-induced thrombocytopenia and thrombosis) is a autoimmune mediated state and can lead to skin necrosis and hemorrhage.

Front 36

Who gets vit K deficiency and what does it lead to?

Back 36

Newborns, pts taking certain abx. Leads to bleeding diathesis characterized by bruising and hemorrhage

Front 37

What is the inheritance of MEN1 and what are the 3 main tumor types?

Back 37

Autosomal dominant. Pancreas/GI (MC is ZES - gatrin-producing gastirnoma -> severe refractory PUD), pituitary, parathyroid hyperplasia.

Front 38

What is management of pt with FAP (familial colonic polyposis?

Back 38

Prophylactic proctocolectomy at time of diagnosis bc risk of cancer is 100%. Autosomal dominant

Front 39

What is the management of a "solitary pulmonary nodule" and what is it? What favors benign vs malignant?

Back 39

Solitary pulmonary nodule = 3cm or less coin shaped lesion in middle to lateral 1/3 of lung surrounded by normal parenchyma. Most are benign; calcification of nodules favors benign lesion. "popcorn" calcification is a hamartoma while "bulls eye" is a granuloma. Management: in low risk pts (>40yo, nonsmoker) - look for old x-ray - if no change for last 12mo, benign - follow with CXR q3mo for 1 year, if no growth leave it as such. If high risk (ex: smoker), start with CT followed by FNA - if lesion is missed, do open-lung bx

Front 40

What is polycythemia vera? What are characteristic features? Tx?

Back 40

Cloncal myeloprolif disease of pluripotent hematopoietic stem cells. Characteristic feature: inc RBC mass, mild granulocytosis, inc platelets, LOW EPO. Reversible moderate HTN often occurs d/t inc blood volume. Inc incidence of PUD (histmaine release from basophils) and gouty arthritis (inc cell turnover). PE: plethoric face and splenomegaly. BM: hypercellular. Tx: phlebotomy to keep Hct <45%

Front 41

What is hairy cell, what does look like on perpiheral smear, dx, tx?

Back 41

aka leukemic reticuloendotheliosis. Type of B cell chronic leukemia. Smear: fine, hairlike, irregular projections. BM may be fibrotic, so BM aspirates are often unsuccessful (dry tap). TRAP stain. Tx: Cladribine (purine analog) - toxic to BM. SE: neuro, kidney damage

Front 42

What are chlorambucil and prednisone used to treat?

Back 42

CLL

Front 43

What is CHOP used to treat?

Back 43

non-Hodgkins lymphoma

Front 44

What is the general ddx for microcytic/hypochromicanemia?

Back 44

1. Iron deficiency (dec intake or inc blood loss) 2. Defective utilization of storage iron (anemia of chronic disease 3. Reduced globuin production (thalassemia and other hB-opathies) 4. Reduced heme synthesis (lead poisoning, sideroblastic anemia)

Front 45

How do iron studies differ in the causes of microcytic, hypochronic anemia (iron def, thalassemias, anemia of chronic disease, sideroblastic anemia)?

Back 45

1. Fe-def (dec serum iron and ferritin, inc TIBC). 2. Thalassemias - nL/high iron and ferritin 3. Anemia of chronic disease - low TIBC, nL iron and ferritin. 4. Sideroblastic anemia - normal/high iron and ferritin

Front 46

What level in blood is increased in thalasemia?

Back 46

HbA2

Front 47

What is measure of ferritin an indicator of?

Back 47

iron reserves

Front 48

What is the clinical presentation, labs/dx, and tx of immune thrombocytopenia (idiopathic thrombocytopenic purpura (ITP))

Back 48

Presentation: may be asympt, mucocutaneous bleeding, ecchymoses and/or petechiae, some follow acute viral infection. Labs/dx: ISOLATED THROMBOCYTOPENIA (<100,000), peripheral smear to exclude other possibilities (TTP), check for Hep C, HIV, DX OF EXCLUSION. tx: Platelets >30,000 without bleeding: observe. Platelets <30,000 or bleeding: steroids (first line), splenectomy or rituximab (second line)

Front 49

What is ITP?

Back 49

autoimmne d/o resulting in inc platelet destruction and inhibition of megakaryocyte platelet produciton due to IgG Ab against platelet membrane glycoproteins. Dec in platelets only

Front 50

What is Bernard-Soulier syndrome?

Back 50

AR disorder due to absent platelet glycoprotein Ib-IX-V, which acts as a receptor for vWF. Usually have MILD dec platelets, circulating "giant" platelets, severe platelet dysfunction, and bleeding out of proportion to the degree of thrombocytopenia

Front 51

What are common types of acquired aplastic anemia?

Back 51

Parvovirus, EBV, drugs, high-dose chemo, radiation

Front 52

What type of anemia does maligannt infiltration of BM cause?

Back 52

Pancytopenia

Front 53

What is dilutional thrombocytopenia due to?

Back 53

Transfusion of several units of PRBC

Front 54

What is DIC, what are labs?

Back 54

Assoc with intravascular activation and consumption of all of the components of the coagulation cascade. Typically have thrombocytopenia, microangiopathic hemolytic anemia, low levels of clotting factors (fibrinogen, factors V and VIII) and prolonged PT and PTT times

Front 55

What does hypersplenism do to platelet count?

Back 55

Causes platelet sequestration in spleen. Platelet count usually remains >30,000 and overall platelet mass and function remain normal. No clinical bleeding

Front 56

What is thrombotic microangiopathy?

Back 56

Formation and deposition of platelet-rich thrombi in the microcirculation and is commonly seen in hematologic disorders such as TTP, HUS, DIC. Thrombocytopenia, microangiopathic hemolytic anemia

Front 57

What is vWD?

Back 57

MC inherited bleeding disorder. Due to impaired action of vWF. PROLONGED BLEEDING TIME and PTT, NORMAL PLATELET COUNT

Front 58

How can you tell the difference between autoimmune hemolytic anemia and hereditary sphereocytosis?

Back 58

Both are vascular hemolytic anemias. AIHA is aquired, hereditary spherocytosis is AD. Neg FHx and +Coombs test suggests AIHA while +FHx and neg Coombs suggests HS. Peripehral smear in both can show spherocytes without central pallor

Front 59

What are PE findings of hemolytic anemia?

Back 59

Pallor, jaundice, splenomegaly.

Front 60

What is the ddx of spherocytes without central pallor on peripheral smear?

Back 60

Autoimmune hemolytic anemia and heredistary spherocytosis

Front 61

What are features of hereditary spherocytosis?

Back 61

Spherocytes without central pallor, strong fhx (AD), positive osmoitc fragility test, neg Coombs test

Front 62

What are features of autoimmune hemolytic anemia?

Back 62

Neg fhx, positive coombs test, spherocytes without central pallor, signs of hemolytic anemia on exam. If Coombs is neg and still high suspicion for AIHA, can do a micro-coombs test.

Front 63

How can further classify autoimmune hemolytic anemia? How tx?

Back 63

Warm aggluitinin (IgG) and cold agglutinin (IgM), and also as primary or secondary. Warm: more common, usually d/t autoimmune disease, treated with steroids, rituximab (CD-20 Ab), splenectomy, or other immunosuppressive drugs. Cold: can be due to infection or autoimmune disease causing intravascular hemolysis. Tx: supportive care (warming body) and RBC trasnfusions

Front 64

What is Paroxysmal nocturnal hemaglobinuria?

Back 64

Defect in cell membrane anchor that leads to complement-mediated hemolysis with a neg Coomb's test but evidence of venous thrombosis and sometimes episodic intravascular hemolysis.

Front 65

What are features of G6PD def?

Back 65

X-linked, causes hemoltyic anemia in which hemolytic episoes may be triggered by drug ingestion. Periphearl smear shows Heinz bodies

Front 66

What are the 3 key principles to use of pain meds in pts with terminal stages of cancer:

Back 66

1. try non-narcotics first unless you are sure pt is in severe pain. 2. don't be afraid to give narcotics. 3. prescribe appropriate amt of meds. Initially treat with short acting, then titrate as needed to achieve complete control. Once dose established, switch to long-acting with short acting for breakthrough pai. Transdermal fentanyl patches take 8-12hrs to take full effect, so not good to start with

Front 67

What should you suspect in smokers who present with Horner's syndrome? (partial ptosis, miosis, anhidrosis)?

Back 67

Lung cancer until proven otherwise. Start with CXR

Front 68

What are usually and additional symptoms of lung cancer?

Back 68

Cough with bloody sputum, SOB, CP, loss of appetite, weight loss. Additional symptoms: weakness, difficulty swallowing, hoarseness or changing voice, facial swelling, facial paralysis, eyelid drooping.

Front 69

What does tamoxifen put you at risk for?

Back 69

Endometrial cancer (arises from lining of uterus) and uterine sarcoma (arises from muscualr wall of uterus) and venous thrombosis bc it has mixed agonist and antagonist activity on ER.

Front 70

What does sideroblastic anemia show in labs?

Back 70

Microcytic/hypochromic anemia stimulating iron-def anemia, but iron stores are elevated and TIBC dec. Results from defective heme synthesis, usually due to pyrixodine-dependent impairment in early steps of protoporphyrin synthesis.

Front 71

What type of anemia can INH cause?

Back 71

sideroblastic (pyridoxine (B6) antagonist)

Front 72

How can treat pt with sideroblastic anemia?

Back 72

if identifiable cause of B6 def (alcohol, drug) can give pyridoxine and easily fix it

Front 73

What type of anemia should suspect in pts taking lots of pain meds?

Back 73

Iron-def anemia due to NSAIDs -> chronic low grade blood loss in GI tract, often the result of PUD.

Front 74

What type of arthritis can be assoc with anemia of chronci disease?

Back 74

RA, but not OA

Front 75

What type of anemia does copper deficiency lead to?

Back 75

Reversible sideroblastic anemia. MC in pts with prolonged history of TPN, celiac disease, or prior gastrectomy

Front 76

What are s/s of Vit C def?

Back 76

aka scurvy. Bleeding gums and ecchymoses secondary to altered collagen syntehsis.

Front 77

What are schistocytes and whend o you see them?

Back 77

(aka helmet cells) - fragmented erythrocytes. Occur in microangiopathic hemolytic anemias (DIC, HUS, TTP) and due to RBC destruction from prosthestic heart valves.

Front 78

What are labs for hemolytic anemia?

Back 78

Dec serum haptoglobin and inc LDH and bili (haptoglobin is a serum protein that bidns to free Hb and promotes its excretion into reticuloendothelial system - in intravsacular hemolysis, the amt of free Hb in serum exceeds binding capacity of haptoglobin -> dec level of haptoglobin.

Front 79

What does hemochromatosisi cause?

Back 79

Cirrhosis, HCC, skin darkening, insulin resistance

Front 80

What is a dec retic count in presence of anemia characteristic for?

Back 80

Aplastic anemia

Front 81

What will happen to retic count in hemolytic anemias?

Back 81

Inc to compensate for inc destruciton of RBC

Front 82

What conditions is dec albumin level assoc with?

Back 82

Cirrhosis (dec production), nephrotic syndrome, protein wasting enteropathy (inc losses)

Front 83

What extracolonic tumor is most common in Lynch syndrome?

Back 83

Endometrial - 43% of females

Front 84

What is hereditary telangiectasia (Osler-Weber-Rendu syndrome)? How can get inc Hct?

Back 84

AD, characterized by diffuse telangiectasias, recurrent epistaxis, widespread AVMs. AVMS tend to occur in mucous membranes, skin, GI tract, but can also occur in liver, brain, lung. AVMs in lung can shunt blood from R to L side of heart, causing chronic hypoxemia and reactive polycythemia -> inc Hct. Can present with massive, sometimes fatal hemoptysis

Front 85

What is the median age for polycythemia vera?

Back 85

60yo

Front 86

How does PHTN and polycythemia coexist commonly?

Back 86

Polycythemia can cause pulmonary PTHN by inc blood viscosity within pulmonary vasculture. Additionally, in pts with chronic hypoxemia, but pulmonary HTN and polycythemia can result

Front 87

What does CO poisoning cause?

Back 87

Dec amt of O2 that can be carried in the body (note: body compensates by developing a secondary polycythemia.

Front 88

What should suspect in pts with new-onset DM, arthropathy, hepatomegaly

Back 88

Hemochromatosis - AR, intestinal iron absorption abnormally increased -> iron deposition in tissues, esp live,r pancreas, heart, pituitary. Can have hepatomegaly, hyperpigmentation, DM, arthropaty, restrictive heart disease, hypogonadism. Can develop liver cirrhosis with inc risk of HCC. Will have high iron, ferritin, and transferrin. Liver bx to confirm dx if iron studies bnormal

Front 89

What can you use in multiple myeloma to look for lytic lesions?

Back 89

Skeletal survey

Front 90

What do you see in periphearl smear of G6PD def?

Back 90

Bite cells and Heinz bodies

Front 91

What will see with LDH in autoimmune hemolysis and how can differentiate from microangiopathic hemolytic anemia?

Back 91

High LDH in both, but will see spherocytes in AIHA and schistocytes in microangiopathic hemolytic anemia

Front 92

What are schistocytes and inc LDH suggestive of?

Back 92

RBC destruction

Front 93

What is Trastuzumab used for and what are SE?

Back 93

(Herceptin). Commonly used to treat breast cancer that is HER2 positive. Can also be used in pts with early stage disease for an adjuvant effect or in pts with later stage mets disease. SE: combo of trastuzumab with chemo can lead to cardiac toxicity -> get ECHO before starting treatment for baselin and to consider other treatments if poor baseline heart function

Front 94

What is Waldenstrom's macroglobulinemia and what are 2 important diagnostic clues? How can differentiate from multiple myeloma?

Back 94

Rare, chronic plasma cell neoplasm. Hyperviscosity of blood owing to excss production of IgM. 2 important diagnostic clues: 1. IgM spike on electrophoresis. 2. Hyperviscosity. Other major s/s of disease: inc size of liver, spleen, and some LN; fatigue due to anemia; tendency to bleed and bruise easily; night sweats; headache and dizziness; various visual problems; pain and numbness in extremities due predominantly to demyelinating sensorimotor neuropathy. Multiple myeloma can have ismilar symptoms, but Ig's are IgG and IgA, not IgM and rarely have hyperviscosity syndromes such as retinal vein engorgement.

Front 95

What is the main Ig involved in heavy chain disease?

Back 95

IgA

Front 96

What is a giant cell tumor of bone, how does it present clinically and on x-ray? What is treatment?

Back 96

Benign and locally aggressive skeletal neoplasm seen in young adults. Present with pain, swelling, dec ROM at involved site. 10-35% have pathologic fractures due to thinning of bone cortex in weight bearing areas. x-ray: in epiphyseal regions of long bones (MC is distal femur and proximal tibia around knee joint) - see osteolytic lesions with a "soap bubble" appearance. MRI can show tumor containing both cystic and hemorrhagic regions. Path: sheets of interspersed large osteoclastic giant cells that appear as round-to-oval polygonal or elongated mononuclear cells. Tx: surgery (interlesional curettage with or without bone grafting) is first line

Front 97

What is a bakers cyst and what does US show?

Back 97

swelling on medial side of popliteal fossa due to enlarged gastrocnemius-semimembranous bursa. Often secondary to DJD or injuyr. US: cystic, fluid filled space

Front 98

What is Osgood-Schlatter disease?

Back 98

Overuse injury caused by repetitive strain. Typically seen in young childrena nd adolescents who have recently undergone rapid growth spurt. X-ray: avulsion of the apophysis of the tibial tubercle

Front 99

What is Osteitis fibrosa cystica?

Back 99

Aka von Recklinhausen disase of the bone. Rare, most commonly d/t hyperPTH from parathyroid carcinoma. Osteoclastic resorption of bone leads to replacement with fibrous tissue (brown tumors) and causes bone pain. Imaging: subperiosteal bone resorption on the radial aspect of the middle phalanges, distal clavicular tapering, "salt and pepper" appearance of the skull, bone cysts, and brown tumors of long bones

Front 100

What is an osteoid osteoma?

Back 100

Sclerotic, cortical lesion on imaging with central nidus of lucency. Typiclaly causes pain that is worse at night and unrelated to activity. Pain quickly releived with NSAIDs

Front 101

What is the initial drug of choice for hyperCa of malignancy?

Back 101

Bisphosphonates (zoledronic acid, pamidronate

Front 102

How do you treat asymptomatic/mild vs severe vs mild hyperCa?

Back 102

Asympt/mild (<12): no immediate tx required, avoid thiazides and lithium use, volume depletion, prolonged bed rest. Severe (>14 or symptomatic): short term immediate tx with normal saline hydration + calcitonin and avoid loop diuretics unless CHF; long term with bisphosphonates. Moderate (12-14) no treatment unless symptomatic - then same as severe.

Front 103

What is the MOA of bisphosphonates?

Back 103

Interfere with osteoclast-mediate bone resorption to inhibit Ca release and can also prevent skeletal complications in pts with bone mets

Front 104

Why need hydration in hyperCa?

Back 104

HyperCa induces urinary salt wasting. Hypovolemia in turn exacerbates hyperCa by impairing the renal clearance of Ca. Thus, pts with mod-severe hyperCa who ahve volume depletion (dry mucous membranes, high BUN) require normal saline at 200mL/hr with goal urine output of 100-150mL/hr

Front 105

What is the initial treatment of SIADH?

Back 105

Fluid restriction. If severely symptomatic or resistant hyponatremia, can give hypertonic saline. Can also use loop diuretics in combo with salt tabs

Front 106

What is a common cause of hypoNa in lung cancer?

Back 106

SIADH (esp small cell)

Front 107

When should consider demeclocycline in SIADH?

Back 107

only if fluid restriction and high salt intake don't correct hypoNa. It acts at renal collecting tubule to decrease responsiveness to ADH, but can be nephrotoxic and rarely necessary

Front 108

What can happen to Ca levels in pts who recieve the equivalent of more than 1 blood volume of blood transfusions or PRBC in 24hrs?

Back 108

Dec bc of elevated plasma levels of citrate which chelates Mg and Ca.

Front 109

What is the standard of care for solitary brain mets with stable extracranial disease? What about multiple brain mets? HY***!!

Back 109

Surgical resection for solitary, palliative whole brain radiation for multiple. Role of surgery is to provide immediate relief from mass effect of tumor, get histologic dx, and improve local control of tumor.

Front 110

What is the most common tyep of intracranial brain tumor?

Back 110

Mets - usually seen at grey-white matter junction. Often seen in non-small cell lung cancer

Front 111

What is the first step in the management of severe symptomatic hyperCa?

Back 111

Vigorous hydration with IV normal saline. In addition, concurrent treatment with calcitonin and bisphosphonates is also recommended to rapidly reduce serum Ca levels. Long term management with bisphosphonates

Front 112

What is the mutation in CML? What is used for treatment?

Back 112

CML - reciprocal translocation of chr 9 and 22 can form Philadelphia chromosome containing the BCR/ABL fusion gene. Tx: tyrosine kinase inhibitors ex: Imatinib (inhibit abnormal BCR/ABL gene)

Front 113

When are retinoic acid receptors targeted?

Back 113

Acute promyelocytic leukemia

Front 114

What should you expect in strict vegetarians with anemia and neurologic complaints? What do you have to do to treat?

Back 114

B12 def. Folic acid may correct the anemia, but won't correct the neurologic complications

Front 115

What are s/s of B12 deficiency?

Back 115

Macrocytic anemia, fatigue, wt loss, constipation, dec appetite, neuropathy

Front 116

What is the screening test for syphillis?

Back 116

FTA-ABS

Front 117

What should you suspect in alcoholic smokwer who presents with palpable cervical LN? What is the best initial test?

Back 117

Squamous cell carcinoma of the mucosa of the head and neck. Best initial test: panendoscopy (EGD, bronchoscopy, laryngoscopy) to detect the primary tumor. After panendoscopy, typically get bx of primary tumor

Front 118

What do you see on peripheral smear in ALL?

Back 118

Inc blasts. Young pts

Front 119

What do you seen on peripheral smear in CLL?

Back 119

Mature small lymphocytes. Elderly

Front 120

What are the lab features of CML?

Back 120

Seen in adults. Leukocytosis, basopihlia, marked splenomegaly.

Front 121

What do you see on peripherla smear of Hodgkins pts?

Back 121

Normal blood smears but see Reed Sternberg cells on LN biopsy

Front 122

What do you see on peripheral smear of folate or B12 def?

Back 122

Hypersegmented nutrophil with large RBC

Front 123

What are the homocysteine and methylmalonic acid levels in B12 and folate def?

Back 123

Both result in elevated homocysteine levels (and dec methionine concentrations). B12 def will have high methylmalonic acid, but will be normal in folate def

Front 124

When are ferritin levels increased and dec?

Back 124

Dec in Fe-def anemia, inc in hemochromatosis, sideroblastic anemia, and acute inflammatory steates

Front 125

What is AFP used as a marker for?

Back 125

Testicular germ cell tumors and HCC

Front 126

When do you see inc urinary VMA?

Back 126

Pts with pheochromocytomas

Front 127

What is the inheritance of hereditary spherocytosis and what is the inheritance pattern?

Back 127

AD. Classic triad of hemolytic anemia, jaundice, splenomegaly. Defect is in Ankyrin gene -> abnroaml RBC plasma memrbane scaffolding proteins. Spherocytes more prone to splenic sequestration.

Front 128

What are pts with hereditary hemochromatosis at risk for?

Back 128

Biliribuin gallstones and parvovirus B19 infection causing aplastic anemia.

Front 129

What will see in labs for hereditary spherocytosis?

Back 129

Elevated mean cell Hb concentration (MCHC) due to memrbane loss and RBC dehydration. Also, reticulocytosis, normal/sl low MCV, elevated RBC distriubtion

Front 130

How do you diagnose hereditary spherocytosis? How do you treat?

Back 130

Made clinically but can confirm wtih osmotic fragility test or eosin-5-maleimide binding test. Tx: supportive with folic acid and transfusions for severe anemia. Splenectomy can improve anemia and reduce gallstone risk, but doesn't change the inc MCHC in RBCs

Front 131

When do you see absent CD55 on RBC membranes?

Back 131

PNH - causes hemolytic anemia induced by the complement system

Front 132

When is HbA2 level incrased? Hb F?

Back 132

HBA2 inc in beta thalassemia trait. Heb F inc in sickle cell, beta thal, congenital aplastic anemia, and hereditary persistence of fetal Hb

Front 133

When should you suspect type 2 HIT?

Back 133

Heparin exposure >5d AND any of the following: Platelet count dec by >50% from baseline, arterial or venous thrombosis, necrotic skin lesions at heparin injection sits, or acute systemic (anaphylactoid reactions) after heparin.

Front 134

How dx and tx type 2 HIT?

Back 134

Dx: Serotonin release assay is gold standard, bust art treatment in suspected cases prior to confirmatory test. Tx: Stop ALL heparin products (including LMWH). Start a direct hrombin inhibitor (ex: argatroban) or fondaparinux (synthetic pentasaccharide). Do all of this even if no thrombus has occured. Don't start warfarin right away (contraindicated bc rapidly dropping protein C levels is prothormbotic) - start after treated with non-heparin anticoagulant and platelet count recovered to >150,000.

Front 135

What is type 2 HIT and what does it lead to?

Back 135

Autoimmune disorder due to Ab against heparin-platelet factor 4 complex. Leads to platelet activation, thrombocytopenia, pro-thrombotic states

Front 136

Do you use prophylactic platelet transfusions to prevent bleeding in HIT pts?

Back 136

Generally avoided due to concerns for causing thrombotic events. Can consider if overt bleeding or undergoing invasive procedures with high bleeding risk, esp if heparin has been stopped for several hours

Front 137

How can you tell the difference between CML and leukemoid reaction? How are they similar?

Back 137

They are indistinguishable on peripheral film - both have sudden elevation of total WBC count and marked inc in granulocyte precursors (L shift). Leukocyte alkaline phosphatase (LAP) test can distinguish - LAP dec in CML, but inc in presence of subsequent secondary infections (leukemoid reaction) --> if suspicious for CML, look for philadelphia chromosome

Front 138

What will happen to pH balance in diarrhea?

Back 138

Metabolic acidosis and mild prerenal azotemia

Front 139

What happens to leukocyte alkaline phosphatase in polycythemia vera?

Back 139

Increased but unlike leukemoid reaction, will also ahve inc RBC mass (with mild granulocytosis and low EPO level).

Front 140

What do the labs look like for secondary polycythemia?

Back 140

ELevation in RBC mass only (rest of cell lines are normal

Front 141

What type of anemia is common after total or patial gastrectomy?

Back 141

B12 def.

Front 142

How does B12 def cause anemia?

Back 142

B12 inhibits DNA synthesis (via inhibiting THF formation)

Front 143

What are common causes of B12 def?

Back 143

pernicious anemia, gastritis, instinal disorders preventing absorption, elderly age, total or partial gastrectomy

Front 144

What is the mechanism of anemia in hereditary spherocytosis?

Back 144

RBC membrane instability

Front 145

What anemias have impaired Hb synthesis as their mechanism of disease?

Back 145

Iron-def anemia, other Hb-opathies like sickle cell and alpha and beta thalassemia

Front 146

What is the MOA of hemolytic anemia fo G6PD def?

Back 146

Impaired glutathione synthesis

Front 147

When does immune-mediated hemolysis occur?

Back 147

When IgG autoAb attach to the RBC membrane and promote their removal from circulation in the spleen

Front 148

What are the lab values in hemolysis?

Back 148

Low Hb, inc indirect bili, inc LDH, dec haptoglobin

Front 149

What is G6PD?

Back 149

X-linked, MC enzymatic disorder of RBC, most common in blacks, asians, mediterranean decesent. Develop hemoltyic anemia after infection, exposure to drugs with ihgh redox potential (primaquine and sulfa drugs), fava beans, or metabolic abnormalities. Interestingly, G6PD levels are often normal during hemolytic eipsode

Front 150

What is difference in prsentation between G6PD def and pyruvate kinase def?

Back 150

Both cause hemoltyic anemia, but PK def not precipitated by same things (ex: not drugs). Also, no bite cells on peripheral smear of PK def

Front 151

What is the manifesation of galactokinase def?

Back 151

Cataracts

Front 152

What will see on peripheral smear of sickle cell?

Back 152

sickle cells or Howell Jolly bodies

Front 153

What are the 3 main causes of macrocytic anemia?

Back 153

1. B12 def (pericious anemia MC; typically have neuro deficits 2. Folic acid def 3. Misc (hypothyroid, severe liver disease, anti-metabolites.

Front 154

What are the labs in macrocytic/megaloblastic anemia?

Back 154

Elevated MCV, elevated MCH, normal MCHC. Peripheral smear: macroovalocyte RBC and hypersegmented neutrophiles. Anisocytosis, poikilocytosis, basophilica stippling. Retic count dec, although BM is hyercellular

Front 155

What are the differences in labs between folic acid def and B12 def?

Back 155

LDH inc in B12, nL in folid acid. Achlorhydria present in B12, absent in folic acid. MethylmalonylCoA in urine present in B12, absent in folic acid. Neuro signs present in B12, absent in folic acid

Front 156

What is the sugar water test used for?

Back 156

PNH diagnosis

Front 157

What type of anemia is lead poisoning?

Back 157

Macrocytic (MCV <80)

Front 158

What are prussian blue staining used for?

Back 158

Suspected sideroblastic anemia

Front 159

What can cause folic acid deficiency? HY!!!

Back 159

Poor diet and/or alcoholism. Can also be causd by some drugs that impair absorption of folic acid (ex: phenytoin, primidone, phenobarb) or antagonize its physiologic effects (methotrexate, trimethoprim)

Front 160

Which lung cancer is most likely to cause hyperCa?

Back 160

squamous cell (sCa++mous). Due to PTHrP -> inc Ca resorption from bones and inc renal Ca resorption from distal tuble. Very serious bc can result form mets involvement in bone and usuaully develops as a late complication of cancer

Front 161

What paraneoplastic syndromes are common in small cell lung cancer?

Back 161

ACTH production, SIADH

Front 162

Where in lung are adenocardinomas usually?

Back 162

Periphery.

Front 163

What type of lung cancer assoc with hypertrophic pulmonary osteoarthropathy?

Back 163

Adenocarcinoma

Front 164

What do you see in labs in anti-phospholipid Ab syndrome?

Back 164

False positive VDRL (neg FTA-ABS), prolonged PTT, dec platelets

Front 165

What should you do for pts with antiphospholipid syndrome in pregnancy?

Back 165

Prophlyaxis with low dose ASA and LMWH to avoid pregnancy loss (Warfarin often used in long term therapy, but contraindicated in pregnancy). APS cane promote arterial and venous thrombosis and resultant tendency toward spontaneous abortions

Front 166

How do you treat primary or secondary syphillis?

Back 166

Benzathine penicillin

Front 167

What are the long terms risks of splenecotmy? HY!!

Back 167

Most feared long term complication is overwhelming sepsis with encapsulated bacteria, most commonly strep pneumo - risk present for up to 30+ years. SHoudl get anti-peumonoccoal, haemophilus, and meningococcal vaccines several weeks before operation and daliy oral penicillin prophylaxis for 3-5yrs after or until adulthood for pediatric pts.

Front 168

What do you see in acute hemolytic crisis of G6pD def in peripheral smear?

Back 168

Bite cells with Heinz bodies

Front 169

How does acute viral hepatitis present?

Back 169

N/V, abd pain, jaundice. Can have fever and confusion. Can have dark urine due to hyperbilirubinemia

Front 170

How does acute glomerulonephritis present?

Back 170

Rapid development of gross or microscopic hematuria, dec GFR, HTN

Front 171

What is thalssemia minor?

Back 171

Heterozygous for beta thal. Typically asymptomatic, peripheral smear shows microcytic traget cells

Front 172

What is the initial managemnet for pt suspected to have esophageal cancer?

Back 172

Barium swallow followed by EGD

Front 173

what are the 2 types of esophageal cancers and what are their highlights?

Back 173

Squamous and adenocarcinoma. In US, incidence of squamous has remained relatively constant, while adenocarcinoma has increased a lot. SCC: assoc with smoking and alcohol. Barrett's is a risk factor for adenocarcinoma

Front 174

What are symptoms of esophageal cancer?

Back 174

difficulty swallowing, regurgitation of food, heartburn, wt loss, vomiting, CP unrelated to eating

Front 175

What cancer is H. pylori assoc with?

Back 175

MALT lymphoma (mucosa assoc lymphoid tissue)

Front 176

What are the features of adenocarcinoma of the lung?

Back 176

Least assoc with smoking. Usually located peripherally and consists of columnar cells growing along the septa. Presents as a solitary nodule and may be detected incidentally. It is the msot common type of lung cancer, even in smokers.

Front 177

What is the most common cause of nutritional folate deficiency in the US?

Back 177

Alcohol abuse

Front 178

What are the hematologic manifestations of alcohol abuse?

Back 178

Folic acid def -> megaloblastic aenmia, iron def from chronic blood loss, anemia of chronic disease, thrombocytopenia, macrocytosis (even before development of anemia)

Front 179

What type of anemia is anemia of chronic disease?

Back 179

normocytic, normochromic

Front 180

What is thiamine def assoc with?

Back 180

Alcoholics. Assoc with Wernicke's encephalopathy

Front 181

What is hemarthrosis and what causes it?

Back 181

Bleeding into a joint usually due to trauma (most common), vascular damage, or coagulopathy (ex: hemophilia). Sponatneous hemarthrosis are so characeristic of hemophilia that they are almost diagnostic.

Front 182

What is the pathophys of HIT?

Back 182

Type 2 HIT: due to heparin binding to platelet factor 4 (PF4), a heparin-neutralizing protein released with platelet acvitaiont. The heparin-PF4 complex then triggers IgG antibodies, which bind to the PF4-heparin complex. This Ab-heparin-PF4 complex then acitvates platelets by binding to the platelet Fc receptor. Thrombocytopenia is due to splenic macrophages prematurely removing the acivated platelet-Ab-heparin-PF4 complexes - the complexes also activate adjacent paltelets taht release procoagulants and cause platelet aggregation, thrombin formation, adn thrombosis. The Ab complex can activate microvascular endothelium to release prothrombotic cytokines (vWF) that simtualte more paltelet release or can injure the vasuclar endothelium to promote more thormbosis. The net result is a simultaneous thrombocytopenia and arterial and/or vneous thrombosis. The thrombus consumes more platelets and worsens the thrombocytopenia.

Front 183

When should suspect HIT?

Back 183

Pts treated/recented treated with heparin who develop unexplained thromboctyopenia (50% dec in platelet count from baseline) or newly diagnosed thrombosis (venous or arterial). HIT can occur with IV or subQ unfractionated heparin, LMWH, or even small amts of heparin (ex: heparin flush or heparin-coated catheter)

Front 184

What is hyposthenuria and what is it assoc with?

Back 184

Inability to concentrate urine in kidney, can result in nocturia. FOund in pts with sickle cell disease, but ais also common, and usually less severe, in pts with sickle cell trait. Thought to result form RBC sickling in the vasa rectase of the inner medulla which impairs countercurrent exchange and free water absorption

Front 185

What kidney problems are pts with sickle cell at risk for?

Back 185

Membranoprolif GN or ESRD due to FSGN. Will have significant abnormalties in urinary sediment, such as RBC casts

Front 186

What is the management of small cervical LN in children and young adlts if asymptomatic?

Back 186

Can be obstereved for node growth or development of symptoms (hard cervical nodes in older pt or smoker would prompt investigation for metastatic caner or oropharyngeal neoplasia)

Front 187

What points LAD to benign vs pathologic?

Back 187

Small, rubbery LN are rarely pathologi and often found in children and young adults. <1.0 cm almost always due to benign process. Cancer nodes tend to be firm and immobile. >2,0cm has inc likelihood of malignancy or granulomatous disease.

Front 188

What is erythromycin used for?

Back 188

Macrolide, among first line agents for strep pharyngitis and CAP

Front 189

What do you treat with acyclovir?

Back 189

HSV, VZV

Front 190

How does beta thal minor present?

Back 190

hypochromic microcytic anemia of moderate severity, nonresponsive to iron supplementatino.

Front 191

What are the 2 severeities of beta thal?

Back 191

Minor - defect in 1 of beta thal genes; modest anemia, Hct 28-40%, MCV 55-75. Major: both beta Hb genes are defective - severe anemia, transfusion dependent at an early age

Front 192

What is MOA of heparin?

Back 192

Activates antithrombin III which in turn inactivates thrombin, factor IXa, and factor Xa

Front 193

What is MOA of ASA?

Back 193

antiplatelet agent that functions by inhibiting COX-1, thereby inhibiting thromboxane A2 synthesis

Front 194

What is MOA of clopidogrel?

Back 194

Antipaltelet, prevents platelet activation by blocking paltelet surface ADP receptors

Front 195

What is MOA of simvastatin?

Back 195

lipid-lowering agent that inhibits HMG-CoA reductase

Front 196

When should you suspect multiple myeloma?

Back 196

Elderly pt with anemia, renal failure, hyperCa

Front 197

How does multiple myeloma present?

Back 197

malignant plasma cell disease which releasees monoclonal proteins, resulting in inc plasma volume. Presents in old age, back pain is most common manifestation. Recurrent infections also common. COmplcations include renal failure, perCa, hyperviscositiy

Front 198

What do labs look like in multiple myeloma? How dx?

Back 198

anemia, inc ESR (often >100), Bence Jones proteins in urine. can have dec WBC. Dx: serum immunoelectrophoresis demonstrates characteristically abnormal M-spike. In most cases, this is due to an IgG Ab produciton by the abnormla plasma cells

Front 199

What type of bone lesions in multiple myeloma?

Back 199

Lytic

Front 200

What type of scan for lytic lesions of bone?

Back 200

Skeletal survey (x-ray)

Front 201

What are clinical features of adrogen abuse?

Back 201

Men: dec testicular formation and sperm produciton, gynecomastia. Women: acne, hirsutism, voice deepending, menstural irregularities. CV: LVH, possible dec HDL and inc LDL. Psych: aggressive beavhior, mood changes. Heme: polycyhemia, possible hypercoag

Front 202

What are s/s of glucoagonoma and how dx?

Back 202

Suspect in pt with mild DM or hyperglycemia with necrotic migratory erythema, diarrhea, anemia, wt loss. Dx: Glucagon >500

Front 203

What is ddx for elevated glucagon?

Back 203

Glucagonoma, hypoglycemia, sepsis, Cushigns, pancreatitis

Front 204

What are s/s of gastrinoma?

Back 204

Abd pain, dirrhea, anemia, wt loss

Front 205

When do you see anti-glutamin acid decarbodxylase ab?

Back 205

type 1 DM

Front 206

What are s/s of carcinoid?

Back 206

Episodic flushing is most characteristic. Diarrhea, wt loss also

Front 207

What is stepwise approach to breaking bad news?

Back 207

1. quiet, private, comfortable environment 2. ask how much pt knows, or what he might then expectations are - "waht do you think of your symptoms" 3. ask how much pt wants to know 4. give him a warning shot - "unfortunately, this is more serious than what i had thought earlier" 5. break bad news if he wnats you to "the results show you ahve lung canceR" 6. give prognosis, but always keep him aware that all of options available to keep him comforatble 7. try to explain clearly and simply

Front 208

What should you suspect in pt with firm, solitary LN in older pt who smokes? What about it if is in head and neck?

Back 208

LN mets. if H&N - squamous cell carcinoma - next step is bx

Front 209

What are LN that are due to infections usually like?

Back 209

Painful

Front 210

What are LN in SLE like?

Back 210

in up to 50% of pts. Soft, discrete, nontender. FOund in axilla, cervical, inguinal regions.

Front 211

what are the most common causes of brain mets?

Back 211

LUNG > Breast > unknown primary > melanoma > colon. Tumors that rarely mets to brain are prostate, esophagel, HCC, oropharyngeal, non-melanoma

Front 212

How does glioblastoma multiforme present?

Back 212

primary CNS tumor arising within the brain parenchyma. Usually presents as a solitary mass with central necrosis and extensive vasogenic edema.

Front 213

What is MCC of brain tumor?

Back 213

Mets

Front 214

What is the differential dx for anterior mediastinal mass:

Back 214

4 Ts: thymoma, teratoma (and other germ cell tumors), thyroid neoplasm, and terrible lymphoma.

Front 215

What do seminomas and nonseminomas do to HCG and AFP?

Back 215

Seminomas: inc HCG, nL AFP. Non-seminomas: inc AFP, many also have inc HCG

Front 216

What are types of nonseminoatous tumors?

Back 216

Yolk sac tumors, choriocarcinomas, embryonal carcinoma

Front 217

What is the most common manifestation of hemophilia?

Back 217

Hemarthroses, esp after only minimal trauma

Front 218

What is the treatment of hemophilia?

Back 218

replace the missing coagulation factor

Front 219

What are the common sites of bleeding in hemophilia?

Back 219

Joints are most common (80%), skeletal muscle, mucous membranes of head and neck (ex: epistaxis), GI, GU

Front 220

What is the common difference in bleeding between hemophilia and platelet dysufnction?

Back 220

Larger bleeding in hemophillia (bruising, hemarthorses). Platelet dysf has smaller bleeding (petechiae, smaller ecchymoses)

Front 221

What do red papules over trunk and lips most likely represent?

Back 221

Cutaneous AV malformations - if diffuse, probably has hereditary telangiectasia, an AD disorder of recurrent epistaxis, telangeictasias, and pos Fhx.

Front 222

Who gets CLL and what are diagnostic features?

Back 222

Elderly pts. Dx features arereally high WBC with L's predominant and smudge cells. Also can have LAD and splenomegaly. As advances, can have anemia and dec platelets as well

Front 223

What is salvage therapy?

Back 223

Treatment for a disease when standard therapy fails, such as radiation therapy for PSA recurrence after radical prostatectomy for prostate cancer

Front 224

What is adjuvant therapy? Neoadjuvant? Consolidation? Induction? Maintenance?

Back 224

Adjuvant: treatment in addition to standard therapy (at the same time. Neoadjuvant: treatment before standar therapy. Consolidation: given after induction therapy with multidrug regimens to further reduce tumor burden. Induction: initial dose of treatment to rapidly kill tumors cells and send pt into remission (>5% tumor burdon). Maintenance: after induction and consolidation (or initial standard tx) to kill any residual tumor cells and keep pt in remission

Front 225

what are lab values in iron def anemia?

Back 225

Low Hb, low MCV (microcytic), low serum iron and ferritin, high TIBC, low transferrin

Front 226

What are lab values of anemia of chronic disease?

Back 226

low serum iron and low TIBC. Transferrin normal or low. Ferritin high bc it is an acute phase reactant that is elevated in many conditions.

Front 227

What are lab values of hemochromatosis?

Back 227

high serum iron, ferritin, transferrin. Low TIBC

Front 228

What is protocol for colonoscopy surveillance for pts with UC?

Back 228

Should begin after 8yrs of diagnosis (possibly longer if only L colon involved) and do it every 1-2 years.

Front 229

What are the 5 warning signs of basal cell carcinoma?

Back 229

1. open sore that bleeds, oozes, or crusts and remains open for 3+ weeks 2. Reddish patch or irritated area 3. Shiny bump or nodule taht is pearly or translucent and is often pink, red, or white. 4. Pink growth with a slightly elevated rolled border and a crusted indentation in the center 5. Scar-like area which is white, yellow, or waxy and often has poorly defined borders

Front 230

What has the highest cure rate for basal cell carcinoma and how do it?

Back 230

Mohs surgery - special type of surgery where microscopic shaving is done with 1-2mm of clear margins are excised. Highest cure rate, but only indicated in pts with high risk features or those with lesions in functionally critical areas - perioral region, nose, lips ears After treatment, new sites of basal cell cancer can occur - therefore, diligent monitoring necessary.

Front 231

What is Trousseau's syndrome?

Back 231

Hypercoagulable state presenting with recurrent and migratory superficial thrombophlebitis at unusual sites (ex: arm, chest). Uusally assoc with an occult visceral malignancy such as pancreas (most common), stomach, lung, or prostate carcinoma

Front 232

What are facotrs that inc risk of pancreatic cacncer?

Back 232

age, smoking

Front 233

What is peripheral septic thrombophlebitis often assoc with and how do pts present?

Back 233

Breaks in skin due to catheters, venipuncture, or IV injections. Present with fever, pain with swelling at infection site.

Front 234

What do you use barium swallow for?

Back 234

To evaluate esophageal abnormalities (dysphagia, odynophagia) or documenting aspiration.

Front 235

What are the most common durgs to cause peripheral neuropathy?

Back 235

Vincristine, cisplatin, taxanes (docetaxel, paclitaxel). Others: phenytoin, INH, amiodarone, hydralazine

Front 236

When do you see extramedullary hematopoiesis (RBC production outside of the bone marrow)?

Back 236

Hematologic maliganncies, chronic anemias (ex: thalassemia, sickle cell), myelofibrosis, idiopathic

Front 237

What are classic findings in multiple myeloma? What do you see in BM?

Back 237

Lytic bone lesions, marrow plasmacytosis, urine and serum monoclonal proteins, lytic lesions, hyperCa, renal failure. Back pain is most common symtpom. Also see recurrent infections. Complications: renal failure, hyperCa, hyperviscosity

Front 238

What do you see in BM in essential thrombocytosis?

Back 238

Inc marrow cellularity with megakaryocytic hyperplasia

Front 239

When do you see hypoplastic fat-filled BM with no abnormla cells?

Back 239

Aplastic anemia

Front 240

When do you see hypocellular and fibrotic BM?

Back 240

myelofibrotic disorders

Front 241

What is pathophys of anemia of chronic disease?

Back 241

iron-trapping within macrophages -> dec serum iron concentrations and poor iron availability for Hb synthesis.

Front 242

What disorders is anemia of chronic disease common in?

Back 242

commonly assoc with chronic inflammatory diseases (ex: infections, cancer, autoimmune) but can also be observed in heart disease, DM, acute inflammation

Front 243

What is treatment for anemia of chornic diseas?

Back 243

Treat the underlying disease

Front 244

What is the definitive treatment for hereditary spherocytosis?

Back 244

Splenectomy

Front 245

What can you use splenectomy for?

Back 245

Definitive treatment for hereditary spherocytosis. Also part of treatment algorithm for ITP and chronic idiopathic myelofibrosis with refractory anemia

Front 246

What is a poor prognostic factor for CLL?

Back 246

Thrombocytopenia

Front 247

What is a classic description of "smudge cells"

Back 247

"leukocytes that have undergone partial breakdown during preparation of stained smear or tissue section bc of their greater fragility"

Front 248

What do you see in labs of CLL?

Back 248

High WBC, can also have splenomegaly, anemia, dec platelets (poor prognosis)

Front 249

What type of L is CLL?

Back 249

B cell disease

Front 250

When should you suspect TTP?

Back 250

Pt with hemolytic anemia and thrombocytopenia. Also can have AMS, renal failure and/or fever

Front 251

How dx TTP?

Back 251

Clinically bc measuring ADAMTS13 is not used right now. SHould get peripheral smear looking for schistocytes (almost all pts wil ahve it)

Front 252

What causes TTP?

Back 252

most are either idiopathic or secondary to drug toxicity. HIV is a risk factor as well.

Front 253

How treat TTP?

Back 253

Plasma exchange

Front 254

When you have autoimmune hemolytic anemia in setting of a malignant lymphoproliferative disorder, what type of AIHA is it? How treat?

Back 254

Warm. Tx: prednisone divided into doses. If ineffective, splenectomy

Front 255

What is the MOA of autoimmune hemolytic anemia?

Back 255

Anti-RBC IgG immunoglobulins cover the cell membranes of erythrocytes and cause their removal from the ciruclation in the spleen's reticuloendothelial system

Front 256

How does CKD cause anemia?

Back 256

Secondary to dec EPO production - these pts benefit from erythropoietin supplementation

Front 257

How can detect overexpression of oncogene HER2? Why does it matter to find out?

Back 257

Immunohistochemical staining or FISH. If positive, predices positive response to trastuumab and ahtracycline chemotherapy

Front 258

What should suspect with hemolytic anemia, venous thrombosis, and diminished hematopoiesis (anemia)?

Back 258

PNH - paroxysmal destruction of RBC bc have no CD55 and CD59 (attacked by complement). Intravascular hemolytic anemia - low Hb, low haptoglobin, high bili and LDH. Tendency towards venous thromboses, esp in hepatic veins. Can have mild thrombocytopenia.

Front 259

How confirm dx of PNH?

Back 259

Flow cytometry - absence of CD55 and CD59 on surface of RBC

Front 260

What is senile purpura?

Back 260

Ecchymoses that occur on elderly patients' extensor surfaces due to perivascular connective tissue atrophy. May be unsightly but not as dangerous and doesn't require further workup

Front 261

What conditions cause poor platelet adhesion?

Back 261

Anti-platelet meds (ASA, clopidogrel, eptifibatide) or vWD. Renal failure can also cause it

Front 262

What is lupus anticoagulatn?

Back 262

IgM or IgG that prolongs the aPTT by binding the phospholipids in the assay. Assoc with inc risk of thrombosis and spontaneous abortion

Front 263

What are the vit K dependent factors?

Back 263

II, VII, IX, X

Front 264

What numbers does vit K def affect?

Back 264

inc PT and aPTT, but PT more affected

Front 265

What is next step in management of pts with bone pain in prostate cancer who have undergone orchiectomy (androgen ablation)?

Back 265

Radation

Front 266

What is flutamide?

Back 266

non-steroidal anti-androgen. When combined with an LHRH agonist, prolongs survival of pts with prostate cancer and limited disease.

Front 267

When can you use bisphosphonates in bony mets?

Back 267

may be useful bc reduced bone sorption. useful in controlling chronic pain from bony mets, but slow onset of action

Front 268

What is a febrile transfusion reaction?

Back 268

Caused by Ab in the patient's plasma reacting with the donor's leukocytes. Can dec probability by leukocyte depletion techniques (like cell washing). Nonhemolytic Fever and chills that respond to NSAIDs and acetaminophen

Front 269

What is acute hemolytic transfusion reaction?

Back 269

Presents with fever, chills. Classic triad of fever, back pain, red/pink urine rarely seen. prevent with careful crossmatching. Rule out by stopping transfusion and getting sample for direct antiglobulin (Coombs) testa nad plasma free Hb. UA will oftne show Hb

Front 270

What should suspect in any pt with history of cancer or fever who presents with back pain and neuro symptoms, esp loss of bowel or bladder function? What is next step?

Back 270

Compression of the thecal sac. Next step is glucocorticoid treatment while awaiting test results to dec risk of permanent neurologic damage. MRI is most common test for dx

Front 271

How does compression of the thecal sac present?

Back 271

Pain is generally first manifestation in pts with epidural SC compression from malignancy. Neuro ysmptoms - imbalance, weakness, bowel/bladder problems. TOC is MRI, but start steroids first while awaiting results

Front 272

What is Leuprolide?

Back 272

Gonadtropin releasing hormone (GnRH) antagonist frequently used in treatment of metastatic prostate cancer

Front 273

What is pica?

Back 273

appetite for nonnutrtive substances such as ice, clay, dirt, paper products. Can be indicative fo iron deficiency

Front 274

What is iron?

Back 274

A mineral, not a vitamin!

Front 275

What is oral leukoplakia?

Back 275

Precancerous white patch or plaque on oral mucosa. 1-20% progress to cancer in 10yrs

Front 276

What are causes of macrocytic anemia (MCV >100)?

Back 276

Folate deficiency, B12 def, myelodysplastic syndromes, AML, drug induced (hydroyurea, zidovudine, chemo agents), liver disease, alcohol abuse, hypothyroid

Front 277

What type of anemia does sickle cell cause?

Back 277

Chromic, normocytic hemolytic anemia with appropriate reticulocytosis response. But, folate def can occur due to inc RBC turnover and dec consumption of folate in BM -> daily folic acid supllementation recommended

Front 278

What is hyperhemolytic crisis?

Back 278

Acute, severe anemia with INC RETIC count in sickle cell disease

Front 279

How can you get iron overload in sickle cell?

Back 279

Due to multiple RBC transfusions

Front 280

What is splenic sequestration in sickle cell disase?

Back 280

Vaso-occlusive and splenic pooling of RBC in sickle cell, which leads to an aburpt and marked fall in Hb.

Front 281

What is next step in iron-def in adult male or post-menopausal female?

Back 281

Test for occult blood in stool, bc chronic GI blood loss is the most common cause (dietary iron def and malabsorption are rare casues)

Front 282

What are GI causes of chroic blood loss?

Back 282

Colon cancer, PUD, angiodysplasia, diverticula, etc

Front 283

How does pernicious anemia present?

Back 283

megaloblastic anemia, atrophic glossitis (shiny tongue), vitiligo, thyroid disease, neuro abnormalities (shuffling broad-basted gait, loss of position and vibration sens). Can als ohave dec platelets or WBC. Vitiligo

Front 284

What is CML?

Back 284

Myeloprolif d/o of uncontrolled production of mature or maturing granulocytes. Present with splenomegaly, anemia, thrombocytosis, leukocytosis with WBC >100,000

Front 285

What are the 2 major SE of INH?

Back 285

Peripheral neuropathy and hepatotoxicity. Can also cause siderobalstic anemia

Front 286

How do myelodysplastic syndromes present?

Back 286

Mostly in adults and present with pancytopenia and macrocytosis (elevated MCV). Can be differentiated from pernicious anemia with peripheral smear and BM for dysplastic cells

Front 287

What test should be done in newly dx myasthenia gravis pts?

Back 287

CT of chest looking for thymoma

Front 288

How does myesthenia gravis present?

Back 288

Can affect any muscle in the body - most common symptoms relate to weakness of muscles that lift up the lid (ptosis) or move the eyes (double vision). May have SOB or dysphagia. NO PAIN OR NUMBNESS.

Front 289

How dx mysasthenia gravis?

Back 289

EMG and Ach-R Ab test. After dx, get CT of chest looking for thymoma

Front 290

When do you use edrophonium (Tensilon) test?

Back 290

Dx MG, but not as sensitive as Ach-R Ab test and hard to interpret

Front 291

What are Anti-Jo Ab for?

Back 291

Polymyositis

Front 292

What are Anti-RNP AB for?

Back 292

Mixed CT disease

Front 293

What should suspect in pt with no prior history of skin disease presents with eczematous rash near nipple that doesn't improve with topical treatments?

Back 293

Paget's disease of breast. Majority of Paget's pts have underlying breast adenocarcinoma

Front 294

How does Pagets disease of breast occur?

Back 294

When severe, ductal carcinoma can infiltrate into the dermal lymphatics with resulting edema, erythema, and warmth of the entire breast that is known as inflammatory carcinoma. When the rash is localized to the nipple and has ulcerating eczematous appearance, the primary consideration should be Paget's disease of the breast. Approx 85% of paget's disease have underlying breast cancer

Front 295

What will skin bx of pagets show?

Back 295

Large cells that apper to be surrounded by clear halos bc the cancer cells retract from adjacent keratinocytes.

Front 296

What is a fibroadenoma of the breast?

Back 296

Benign breast lesion.

Front 297

How does breast papilloma present?

Back 297

Often intraductal and can present with a palpable mass and/or nipple discharge

Front 298

Are SCC and lymphoma common in the breast?

Back 298

no

Front 299

What does Prussian blue stain for?

Back 299

Presence of hemosiderin which is found in urine during hemolytic episode

Front 300

What blood problem can alpha-methyldopa and penicillin cause?

Back 300

Autoimmune hemolysis

Front 301

What is low leukocyte alk phos with high WBC classic for?

Back 301

CML. Inc WBC, anemai, inc mature granulocyte forms (segmented neutrophils, bands)

Front 302

How does CML present?

Back 302

>50yo, fatigue, malaise, low grade fever, anorexia, wt loss, bone pains, night sweats and fever. BM: hypercellular with prominent granulocytic hyperplasia. Leukocyte alk phos low

Front 303

What disease can cause low leukocyte alk phos? High?

Back 303

Low leuko alk phos: CML, hypoP, PNH. High: leukemoid reaction

Front 304

What are characteristic signs of CML?

Back 304

Philadelphia chromosome, low leukocyte alk phos, high WBC

Front 305

When do you see no EPO in urine?

Back 305

polycyehtmia vera

Front 306

When do you see auer rods?

Back 306

AML

Front 307

When do you see tartrate resistant acid phosphatase?

Back 307

Hairy cell leukemia

Front 308

What drug can you use to increase appetite and weight gain in pts with cancer-related anorexia/cachexia syndrome?

Back 308

Progesterone analogs (megestrol acetate, medroxyprogesterone acetate) and steroids, but progesterone analogs are preferred due to fewer SE. Tetracyclic antidepressant mirtazapine (Remeron) also shown to help

Front 309

What is the drug of choice for treating and preventing chemo-induced nausea and vomiting?

Back 309

Serotonin antagonists that target the 5HT3 receptor. Ondansetron in thsi group. Sometimes combine with steroids for better effect

Front 310

What is the primary anticholinergic agent used to treat vomiting?

Back 310

Scopolamine - usually in the form of a patch to help reduce motion sickness. Doesn't work that well for chemo-induced symptoms.

Front 311

What antidopamine agents used for N/V?

Back 311

Metoclopramide and prochlorperazine, but not as good as zofran (serotonin antagonist) for chemo-induced vomiting

Front 312

What are motilin receptor agonists used for?

Back 312

Promote GI motlity. ERythromycin's primary use is as antibiotic, but aslo functions as motilin R agonist and can help treat nausea secondary to gastroparesis.

Front 313

How does ectopic ACTH syndrome caused by rapidly growling malignant tumor present?

Back 313

Generally causes HTN, hypoK, metabolic alkalsos, pigmentation without characterstic features of Cushings. When cortisol produced in excess amounts, results in mineralocorticoid effects by bypassing the enzyme 11 beta hydroxyl dehydrogenase. Ectopic ACTH caseus more severe HTN and hypoK

Front 314

What cancers is ectopic ACTH produciotn most assoc with?

Back 314

small cell lung cancer, carcinoid (bronchial, pancreatic, thymus). Not inhibited by cortisol and magnitude of ACTH elevation and rapidity of onset determine the clinical presentation

Front 315

What hematologic abnormalities are common in SLE?

Back 315

Pancytopenia - usually indicates concurrent peripheral immune-mediated destruction of all 3 cell lines. Anemia is most commonly due to anemia of chronic disease, CKD (low EPO from lupus nephritis), Fe-def anemia due to NSAIDs, and autoimmune hemolytic anemia.

Front 316

When blood cells are sequestered and destructed in spleen, what do you see?

Back 316

Pancytopenia and splenomegaly. More common in cirrhosis, malaria, sickle cell

Front 317

When do you more often see ineffective hematopoiesis?

Back 317

Thalassemia and myelodysplastic sydnromes

Front 318

What test is important in the diagnosis of hairy cell leukemia?

Back 318

TRAP (tartrate-resistant acid phosphatase)

Front 319

What will you see on staining in hairy cell leukemia?

Back 319

B cell chronic leukemia. Fine, ahir-like irregular projections from L's. BM can become fibrotic -> dry tap. Cytochemical features: TRAP stain, CD11c marker. Presents with pancytopenia and splenomegaly

Front 320

What do you see in CML?

Back 320

Splenomegaly, neutrophilic leukocytosis (L shift), basophilia, low leukocyte alk phos

Front 321

When do you see Reed-sternberg giant cells?

Back 321

ie Owl's eye cells. Diagnostic for Hodgkins - see them in BM, not peripheral smear

Front 322

What should think of in pt with enviornmental risk factors and headache, nausea, dizziness?

Back 322

CO poisoning

Front 323

How does CO poisoning present?

Back 323

polycythemia, nausea, dizziness, headaches. CO-bound Hb significantly decreases the blood's O2 cparrying capasity - to compensate for dec O2 delviery, body increases RBC production (-> polycythemia)

Front 324

What cell lines are affected in polycythemia vera?

Back 324

All 3 cell lines, esp RBC

Front 325

How do PHTN and polycythemia often go together?

Back 325

Polycythemia itself can cause PHTN by inc blood viscosity within the pulmonary vasculature. In other instances, polycythemia occurs in response to PHTN, esp when the PHTN is related to chornic hypoxemia.

Front 326

How can AV shunting cause polycythemia?

Back 326

Many ways - for example, L->R intracardiac shunt can inc pulmonary blood flow -> PHTN. WHen PHTN severe enough, secondary polycythemia can develop. IN addition, R->L intracardiac shunting can cause chronic hypoxemia, to which the body responds with a compensatory inc in EPO production.

Front 327

Do pts with COPD have anemia or polycythemia?

Back 327

Polycythemia

Front 328

How do you treat MALT lymphoma without mets?

Back 328

Eradication of h pylori with abx + PPI. Chemo only if doesn't work

Front 329

What is management of fibrocystic disase?

Back 329

Treat with aspiration of fluid, which should yield clear yeast and result in disappearance of mass - after, observe for 4-6 weeks. Don't need to send fluid for cytology unless bloody or foul smelling

Front 330

What are s/s of fibrocystic breast mass?

Back 330

Rubbery, firm, mobile and painful mass in yount pt that is more tender during menses. Aspiration of cyst yeild clear fluid and results in disappearance of mass

Front 331

Is mammogram useful in women <35yo?

Back 331

No bc dense bresast tissue doesn't allow for adequate visualization of any masses, if present

Front 332

What is mechanism of anemia in lymphoproliferative disorders such as leukemia and lymphoma?

Back 332

Due to replacement of RBC progenitor cells with cancer cells in BM. Treatment of malignancy may improve anemia, but many chemo agents are myelosuppressive

Front 333

Is autoimmune hemolytic anemia of pts with CLL intravascular or extravascular?

Back 333

Extravascular bc spleen and reticuloendothelial system are considered extravascualr

Front 334

In pts with stroke from sickle cell, what is the next step in treatment

Back 334

Exchange transfusion to dec percentage of sickle cells and prevent second infarct from occurring.

Front 335

Why do pts with sickle cell get strokes?

Back 335

SEcondary to sludging and occlusion in the cerebral vasculature. Ischemic. Not a true thrombus, so thrombolytics and/or heparin/warfarin aren't helpful

Front 336

How to confirm the dx of squamous cell carcinoma of skin?

Back 336

Punch biopsy. Treatment is surgical removal

Front 337

WHen should suspect SCC of skin?

Back 337

Chronic scar that develops into a nonhealing, painless, bleeding ulcer. Involves changes in cells of middle portion of epidermal skin layer. Burns are higher risk. Usually initially painless, then painful with development of ulcers taht dont' heal. Tar derivates also RF, as well as immunosuppression

Front 338

How does polycythemia from OSA occur? How is it treated?

Back 338

Hypoxia-induced increases in EPO (kidneys sense hypoxemia). Tx: treat OSA and polycythemia will improve.

Front 339

What happens to RBC count in CO poisoning?

Back 339

Polycythemia (secondary) due to dec in blood's O2 carrying capacity

Front 340

What is treatment of pts with beta thal trait?

Back 340

Usually asymptomatic with mild anemia, disproportionately high RBC count, low mean MCV, and Hb >10. Peripheral smear shows hypochromia and poikilocytes, including target cells

Front 341

How can differentiate iron def anemia with beta thal minor?

Back 341

Iron def has low RBC count and rarely becomes microcytic until Hb <10.

Front 342

How is RDW different in Fe def and minor thals?

Back 342

High in Fe def, nL in alpha and beta thal minor

Front 343

WHen do you see target cells on smear?

Back 343

alpha, beta thal

Front 344

What happens to iron and ferritin in thalassemias?

Back 344

normal-increased

Front 345

What is Hb on electrophoresis in fe def, alpha thal minor, beta thal minor?

Back 345

Normal in all except beta thal minor, which shows elevated HbA2

Front 346

What is deferoxamine and what is it used to treat?

Back 346

Iron chelator - treats iron overload states in those with transfusion-dependent thalassemia major or sickle cell disease

Front 347

When is EPO the treatment of choice?

Back 347

Anemia of chronic disease, anemia assoc with CKD, and transeitn BM failure after chemo or BM transplantation

Front 348

When is folic acid supplementation used?

Back 348

Megaloblatic anemia due ot folate deficiency, severe forms of hemolytic anemia (ex: heredtiary spherocytosis, PNH, sickle cell), and beta thal major due ot inc demand for erythropoeisis

Front 349

When is hydroxyurea used?

Back 349

To dec frequency and severity of pain crises in pts with sickle cell by increasing Hb F.

Front 350

What is the TOC for pts with autoimmune hemolytic anemia?

Back 350

Prednisone

Front 351

When is splenectomy the TOC?

Back 351

moderate, symptomatic, hereditary spherocytosis. Also used as a treatment option in AIHA and for those with massive splenomegaly assoc with beta thal major or Hemoglobin H disease

Front 352

What is treatment of febrile neuropenia?

Back 352

Start on empiric broad-spectrum Abx ASAP after blood cultures obtained. Empiric monotherapy with anit-psuedomonal agent (ex: cefepmine, meropenem, pip-tazo) is recommended for initial management. Vanco added for those with probable catheter-related infection, soft tissue/skin infection, PNA, or sepsis. No role for CSF as adjunct.

Front 353

What is the definition of febrile neutropenia?

Back 353

A single temp of >38.3 or a sustained temp >38 for >1 hour in a neutropenic pt (ANC <1500).

Front 354

What are the classifications of neutropenia?

Back 354

Mild (ANC <1500), moderate (ANC <1000), severe (ANC <500). All require abx, but both moderate and severe require hospitalization for IV abx. Most common organisms are gram neg, esp pseudomonas but also gram+ sometimes.

Front 355

What does the combo of arterial/venous thrombosis and dec platelets in pt recieving heparin suggest?

Back 355

HIT - Ab against heparin-platelet factor-4 complex are responsible

Front 356

What is non-immune platelet degradation characteristic for?

Back 356

Microangiopathic anemias

Front 357

What is DIC characterized by?

Back 357

Dec platelets, thrombosis, and/or bleeding. Fibrin degradation products are typically seen.

Front 358

How can venous thromboembolism lead to stroke?

Back 358

if pt has persistent patent foramen ovale

Front 359

In pt with CKD who is on EPO, what should you also add?

Back 359

Iron supplementation bc can get EPO-induced surge in RBC production that can precipitate iron-def state

Front 360

What type of anemia do pts with CKD develop?

Back 360

Normocytic, normochromic hypoproliferative anemia due to dec EPO production by failing kidneys

Front 361

How do adult pts get lead toxicity? What are symptoms? Dx, Tx?

Back 361

Occupational expsoure (battery manufacturing, plumbing, mining, painting, paper hanging, auto repair). Pts present with nonspecific symptoms (fatigue, irritability, insomnia), myalgias, HTN, nephropathy, neurocognitive deficits, peripheral neuropathy. Acute expsure can have abd pain, constipation Dx: Blood lead levels, CBC with blood smear, x-ray fluorescence to measure bone lead concetration. Tx: Chelation therapy

Front 362

What type of anemia does lead poisoning cause and what does peipheral blood smear show?

Back 362

Microcytic anemia. PS: basophilic stippling (but can be seen with thalassemias, sickle cell, sideroblastic anemia.

Front 363

What are rare but deadly SE of some vaccinations?

Back 363

Guillian-Barre, acute disseminated encephalomyelitis

Front 364

How does the Schilling test work?

Back 364

Differentiates dietary def from pernicious anemia and malabsortion as causes of B12 def. In dietary, oral radiolabeled B12 is absorbed by the gut and excreted by kidney in normal amounts (they are able to absorb it). If it had been low, would have to differentiate between pernicious anemia and malabsorption - give B12 with intrinsic factor - if normal excretion, then diagnostic of pernicious anemia.

Front 365

What blood tests are elevated/dec in HIT?

Back 365

Dec platelets, high PTT (due to thrombus consumption)

Front 366

How can tell if HIT caused by unfractionated heparin or LMWH?

Back 366

Unfractionated with prolong PTT, whereas LMWH won't

Front 367

Who can macrovascular hemolysis cause microcytic anemia in?

Back 367

Pts with heart valves or severely calcified aortic valves. inc retic count, inc LDH< dec aptoglobin. macrovascular traumatic hemolysis. Peripheral smear: helmet cells or fragmented RBC

Front 368

Who is warm, ab-type autoimmune hemolytic anemia most commonly seen in?

Back 368

Women with some underlying disease affecting the immune sytem (lymphoid neoplasm, collagen vascular disease, congenital immunodeficiency disease. +Coomb's test.

Front 369

What are common causes of ITP?

Back 369

ITP: dec platelets without anemia or leukopenia. Dx of exclusion. Oftne caused by HIV - all pts with presumed ITP should be tested for HIV and Hep C. Also rule out pseudothrombocytopenia due to platelet clumping by EDTA, abciximab, or inadequate anticoag of blood smaple. May need BM bx if neg tests and unexplained

Front 370

What is the rapid plasma reagin test?

Back 370

Nontreponemal Ag test used to screen for syphilis. Congenital syphilis can be assoc with stillbirth, anemia, thrombocytopenia.

Front 371

What lung cancer is SIADH often assoc with?

Back 371

Small cell. Chemosensitive (often)

Front 372

How can diagnose CLL?

Back 372

Peripheral smear - smudge cells. But usually for confirmation of dx, flow cytometry used - prove clonality of abnroaml lymphocytes

Front 373

What is presentation of CLL?

Back 373

Usually >70yo, asympt. Most pts have LAD. Pts are at risk for developing recurrent infections

Front 374

What is the JAK2 mutation assoc with?

Back 374

Myeloproliferative diseases, esp polycythemia vera.

Front 375

What can serum protein electrophoresis detect?

Back 375

Monoclonal protein, as seen in multiple myeloma

Front 376

What is s/s of hereditary spherocytosis?

Back 376

AD disorder of spectrin that leads to inc RBC membrane fragility. +FHx, splenomegaly, spherocytosis, can cause cholecystitis due to pigmented (calcium biliruniate) gallstones

Front 377

What is Gaucher's disease?

Back 377

Can result in anemia, thrombocytopenia, HSM. Pts have painful cortical bne lesions. Gaucher clels with eccentric nucleoli and PAS+ inclusions are diagnostic.

Front 378

What are lab results in lupus anticoagulant?

Back 378

PTT normal or slightly prolonged (prolonged was the question), vWF, BT, platelet count normal. D-dimer normal or high (D-dimer typiclaly elevated in presence of blood clots)

Front 379

What should consider in pt with clotting disorder and prolonged PTT?

Back 379

Lupus anticoagulant/anti-PL Ab - prothrombotic Ig that causes a spuriously prolonged PTT

Front 380

Why are pts with multiple myeloma at inc risk for infection?

Back 380

Due to both total dec in functional Ab and leukopenia that devleops as the BM is filled with malignant plasma cells

Front 381

What is the classic presentation of multiple myeloma?

Back 381

CRAB: hyperCa, renal impairment, anemia, bones (bone pain, lytic lesions, fractures).

Front 382

How do pts with multiple myeloma get kidney disease/

Back 382

IgG Ab or paraproteins produced by myeloma cells can collect in kidney causing renal failure - "myeloma kidney"

Front 383

What happens to protein gap in multiple myeloma?

Back 383

Higher protein gap - paraproteins made by myeloma plasma cells contribute to the total serum protein count but not to the albumin cough - so while normally TP-albumin = 3-4, in MM it is higher.

Front 384

What is the effect of complement deficiencies?

Back 384

Can't effectively opsonize pathogens, predisposing them to recurrent infections, esp with encapsulated bacteria. Complement def are relatively rare.

Front 385

What happens if deficient in any component of MAC?

Back 385

Defective baterial lysis. Type of complement deficiency. Recurrent infections, esp Neisseria

Front 386

What is a granulocyte's bacterial activity attributed to?

Back 386

Products of oxidative metabolism generated intracellularly.

Front 387

What does high homocysteine level put you at risk for and how do you treat it?

Back 387

Inc risk of venous thrombosis as well as atherosclerosis. Involved in B12, B6 and folate metabolism. Treatment with B6 and folate.

Front 388

What is the MOA of clopidogrel and ticlopidine?

Back 388

Inhibit platelet aggregation by blocking ADP receptors on the platelets.

Front 389

What is Clopidogrel used for?

Back 389

Treatment of arterial thromboses (MI, stroke, etc), not venous thromboses

Front 390

When do you use thrombolytics in DVT?

Back 390

Limited to pts with extensive iliofemoral involvement

Front 391

How do you differentiate MGUS from multiple myeloma?

Back 391

MGUS doesn't have renal insufficiency, hyperCa, anemia, or lytic bone lesions. MGUS has 1% risk per year of progression to multiple myeloma. On protein electrophoresis (SPEP), MGUS pts have <3 M proteins with <10% plasma cells in BM whereas MM have >3g/dL M proteins with >10% plasma cells

Front 392

When would consider abdominal fat pad biopsy?

Back 392

If strong concern for amyloidosis.

Front 393

When would serum viscosity study be preformed?

Back 393

FIndings suggestive of hyperviscosity syndrome (blurry vision, nasal bleeding headache)

Front 394

What is the typical scenario for a pt with vit K def (adult)? Labs?

Back 394

Pt who has been kept NPO for a prolonged period of time and recieving broad-spectrum abx. Labs: prolonged PT and PTT (PT more elevated by PTT). Tx: parental administration of Cit K rapidly restores stores in 8-10hrs. FFP indicated for acute hemorrhage

Front 395

What is TTP?

Back 395

Rare form of consumptive thrombocytopenia causd by widespread plaetlet thrombi that form in microcirculation. Thrombocytopenia, microangiopathic hemolytic anemia, fever, neurologic signs characterize it.

Front 396

What is HUS and how can distingusih from TTP?

Back 396

HUS - often follows diarrhea. HUS and TTP both have dec platelets, microangiopathic hemoltyic anemia, and fever. but instead of neurologic signs in TTP, HUS will have renal failure

Front 397

What are labs in DIC?

Back 397

Dec platelets, prolonged PT, PTT, thrombin time. Dec fibrinogen, elevated fibrin degradation products

Front 398

What kind of disorders are factor VIII and IX def and what are labs?

Back 398

X-linked inherited plasma coagulation disorders. Prolonged PTT. Manifests early in life. Impossible to distinguish between the two clinically

Front 399

What are labs in vWD?

Back 399

Inc bleeding time and PTT. PT normal