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79 Cards in this Set

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Protamine sulfate
- positively charged basic protein that inactivates heparin, which is neg charged
- inactivation stops heparin overdose
Aminocaproic acid
- competitively blocks plasminogen activation
- used to treat bleeding induced by fibrinolytic agents
- low molecular weight heparin that potentiates antithrombin III
- can cause bleeding and thrombocytopenia
- fibrinolytic drug
- converts plasminogen to plasmin, which degrades the clot
which condition is associated with bcl-2?
- follicular lymphoma
- bcl2 inhibits apoptosis
- (14:18) translocation
- follicular lymphoma invovles the lymph nodes and bone marrow of older people, and is characterized by slow but relentless growth
- resistant to therapy b/c of ow mitotic activity
what chr abnormalities are found in patients with ALL?
- hyperdiploidy
- philadelphia chr t(12:21)
- t(4:11)
- a neoplasm of pre-B cells that affects children
Burkitt lymphoma
- african form: associated with EBV
- also, the sporadic nonendemic and AIDS-associated forms
- c-myc oncogene (t(8:14)) causes over expression -> nuclear transcription protein -> sustained cell replication
genetics of Multiple myeloma
- 25% of cases have translocations that lead to overexpression of FGFR3- a receptor for fibroblast growth factor implicated in control of cell proliferation
small lymphocytic lymphoma
- B cell neoplasm
- equivalent to CLL
- the neoplastic cells look like normal mature B cells that have CD5
how are PT and PTT affected in DIC? What causes DIC?
- both are increased
- DIC can be seen in obstetric complications: STOP Making New Thrombi
- Sepsis, Trauma, Obstetrics, acute Pancreatitis, Maligancy, Nephrotic syndrome, Thalassemia
what happens to PT and PTT during vit K deficiency?
- both increase
how does vWD affect PT, PTT, Fibrin degredation products, and template bleeding time?
- vWD impairs platelet adhesion -> increase the template bleeding time and the PTT (Intrinsic pathway)
nl hemoglobin values
Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
nl Mean corpuscular volume
80-100 um3
what disorder can cause iron deficiency anemia?
- Uterine leiomyomas
- manifest with pain and abundant blood loss during menstruation
- chronic blood loss -> depressed reservoir of iron
- chronic blood loss is the most common cause of Iron deficiency anemia in western countries
what do you see in lab values for b-thalassemia?
- accumulation of iron
- normal or increase plasma iron and ferritin, and reduced transferritin
how do you differentiate anemia of chronic disease from iron deficiency anemia?
- anemia of chronic disease is associated with increased iron stores -> elevated serum ferritin, reduced transferrin, and increased iron deposits in bone marrow
how do you visualize Hairy cell leukemia cells?
- Tartrate-resistant acid phosphatase (TRAP) stain
- Hairy cell leukemia is a rare B cell lymphoproliferative syndrome, seen most often in older men
what is Chromium 51 used for?
- used to measure red cell survival
what do you a myeloperoxidase stain for?
- used to stain neutrophils
- myeloperoxidase deficiency is clinically similar to chronic granulomatous disease
what is nitroblue tetrazolium used for?
- used to evaluate granulocyte phagocytic function in chronic granulomatous disease
what clotting defect can cause prolonged bleeding times after tooth extraction
- deficiency of vWF leads to failure of platelet adhesion -> poor plug formation -> primary hemostatic bleeding and prolonged bleeding time
- functional deficiency of factor 8 -> secondary hemostatic bleeding and prolonged PTT
when do you see abnormal platelet morphology?
- infiltration of the BM by tumor or fibrosis (myeophthisic anemia), or after splenectomy
when do you see defects in primary platelet aggregation?
- Glanzmann's thrombasthenia
- defect or deficiency in the glycoprotein GpIIb-IIIa complex
- or, aspring induced acetylation and inactivation of COX-1/2 -> failure to synthesize TxA2
when do you see defects in platelet vesicle release?
- storage pool disease and aspirin
what happens to the platelet count during DIC?
- decreases
how does streptokinase bust clots?
- binds and cleaves plasminogen -> producing plasmin
- plasmin directly cleaves fibrin
- the same mech used by tPA
how is antithrombin III diferent from plasminogen?
- antithrombin II is a COAGULATION inhibitor that binds and inactivates thrombin. it is not a FIBRINOLYTIC
- anticoagulant protein taht binds to thrombin and diminishes its capacity to activate fibrinogen, Factor 5, and platelets
- has no fibrinolytic activity
descibe the clinical outcomes of patients with hereditary spherocytosis
- spherocytes become trapped in the splenic cords and are destroyed by phagocytosis -> hemolytic anemia, jaundice, and splenomegaly
- common complications: bilirubin gallstones
when can patients develop hemorrhoids?
- exacerbated in patients with portal hypertension, particularly in patients with hepatic disease like liver cirrhosis
when can Plasmodium vivax and P. ovale cause relapse of clincial symptoms?
- both organisms are capable of causing relapse following recovery from the primary infection b/c both can leave dormant forms in the liver: hypnozoites.
- note: P. falciparum and P. malariae do not possess these forms, can can't cause relapses
- treated with primaquine, which destroys the hypnozoites
P. falciparum and P. malariae and 'recrudesce'
- looks like relapse, but is due to inadequate clearing of the parasites by drugs in the first place
- relapses and recrudences look the same clincially
lymphogranuloma venereum
- initial papular lesion on penis
- followed by multiple enlarged, abscessed lymph nodes draining through the skin : abscessed lymph nodes are called 'buboes'
- causative agent: chlamydia trachomatis
what molecules are mostly likely to be found as Bence-Jones proteins in patients with multiple myeloma?
- VkCk
- most human Igs have the kappa light chain -> most Bence Jones proteins are kappa light chains: variable region of the kappa light chain followed by the constant domain of the kappa LC
- second most common: lamda light chains
which Igs are associated with warm, and which with cold agglutinin?
- IgG: Warm weather is GREAT!
- IgM: Cold ice-cream... MMMM
what are cold agglutinins triggered by?
- mycoplasma pneumonia infection
- infectious mononuclosis
name some drugs that can decrease the metabolism of warfarin and cause a prolonged bleeding time (increased INR)
- norfloxacin
- macrolides: erythromycin, metronidazole
- sulfonamide antibiotics
- first generation cephalosporin that can be used in the treatment of acute cystitis
- urinary tract analgesic that changes the color of urine to a bright orange/red color
what do you see in the peripheral blood smear of a patient with hairy cell leukemia, other than the hairy cells?
- unlike most other forms of leukemia, you see pancytopenia rather than increased numbers of circulating cells
- usually affects middle-aged men, who present iwth prominnet splenomegaly and pancytopenia
how do you treat hairy cell leukemia?
- dramatic response to:
- a-interferon
- 2-chlorodeoxyadenosine
- deoxycoformycin
where do you see Birbeck granules?
- Langerhans cell histiocytosis (histiocytosis X)
- caused by langerhans cells from the monocyte lineage that infiltrate the lung
- primarily affects young adults
- worse with smoking
when do you see elevated leukocyte alkaline phosphatase?
- myeloid metaplasia
- aka chronic idiopathic myelofibrosis (agnogenic myeloid metaplasia, myelofibrosis with myeloid metaplasia)
- extensive extramedullary hematopoiesis
when do you see the philadelphia chromosome?
- occasionally ALL
what can polycythemia vera progress to?
- myeloid metaplasia with myelofibrosis
- there are 4 types of myeloproliferative disorders:
1. CML
2. polycythemia vera
3. myeloid metaplasia with myelofibrosis (MMM)
4 and essential thrombocythemia
- both CML and polycythemia vera are characterized by hypercellular BM producing all three cell lines
- after many years, the BM can burn out -> marrow space is replaced by fibrosis -> hematopoiesis moves to extramedullary sites (spleen and liver)
what is myelodysplastic syndrome (MDS)?
- myeloid dysorder feature maturation defects and ineffective hematopoiesis
- causes cytopenia and acute leukemias
- contrast to myeloproliferative disorders that produce polycythemia and chronic leukemia
how is the haptoglobin-hemoglobin complex cleared?
- haptoglobin is a serum protein that promptly binds to free Hb in the serum, forming a tight complex
- the complex is cleared quickly by the reticuloendothelial system, which allows the Hb to be salvaged instead of being lost in the urine
methemoglobin and DIC
- increases in DIC
- methemoglobin is an oxidized form of Hb
what does the starry sky pattern look like?
- lymphoma composed of small lymphocytes with interspersed macrophages surrounded by clear spaces
- indicated for moderate to severe pain associated with RA, not for muscle soreness
what are you at risk for uric acid kidney stones after chemotherapy?
- increased production of uric acid from purine breakdown during periods of active cell proliferation
- hydration and diuresis is generally instituted after the diagnosis of ALL is made
what can cause cholesterol gallstones?
- DM
- obesity
- pregnancy
- OCPs
- Celiac disease
what is one complication of warfarin?
- warfarin is used for prophylaxis of thromboembolic complications associated with cardiac valve replacement and afib (coumadin)
- can cause skin necrosis on breasts, thighs, and buttocks between 3-10 days of therapy
- caused by warfarin's ability to deplete protein C -> hypercoagulable state -> thrombosis in cutaneous microvasculature
- first generation cephalosporin
- commonly used as a perioperative prophylactic agent
what is one side affect of heparin?
- thrombocytopenia
what is a side effect of vancomycin?
- if administered too quickly, can cause a maculopapular rash on the chest and extremites
- rash disappears once therapy is discontinued
what cell surface markers are on Reed-Sternberg cells?
- CD30, a lymphoid activation antigen
- CD15, a granulocyte antigen
nl mean corpuscular Hb (MCH)
nl mean corpuscular Hb concentration
describe the MCV, MCH, and MCHC levels for megaloblastic anemia
- increased MCV
- increased MCH
- normal MCHC
describe the mechanism of warfarin
- anticoagulant that interferes wtih the synthesis of Vit K dependent clotting factors: 2, 7, 9, 10
- tetratogen: fetal hemorrhage, embryopathy, optic atrophy, brain abnormalities
how is APTT different from bleeding time?
- APTT refers to dysfunction of the coagulation process whereas bleeding time indicates platelet dysfunction
when do you see a 'crew haircut' x-ray?
- sickle cell anemia
- extramedullary hematopoeisis
50 yo pt with fatigue, weight loss
Peripheral smear and bone marrow biopsy shows leukemia of cells in the neutrophil, eosinophil, and basophil lineages
No blast forms
- clonal proliferation of myeloid stem cells, the precursor cells of erythrocytes, granulocytes, monocytes, and platelets
- it's one of the myeloproliferative syndromes
clinical presentation of nodular sclerosis subtype of Hodgkin disease
-painless lymphadenopathy
- night sweats
- generalized pruritis
- fibrotic bands divide the node into multiple nodules
- usually affects children and young adults
Idiopathic thrombocytopenic purpura (ITP)
- autoimmune based
- acquire thrombocytopenia secondary to splenic destruction of opsonized platelets
- usually follows a viral URI or exanthem
- acute form is explosive but self-limited
- chronic form in adults may respond to steroid therapy or splenectomy
define exanthem
eruption on the skin occurring as a symptom of a disease
Bernard-Soulier sydnrome
- platelets can't adhere to collagen
- lack of platelet surface glycoprotein GPIb-IX-V needed for platelet adhesion
- associated with functional defect in platelets- don't aggregate
- aka Glanzmann thrombasthenia
Thrombotic thrombocytopenic purpura (TTP)
- platelets are consumed in intravascular platelet microthrombi
- platelet-derived hyaline microaggregates in small vessels, thrombocytopenia, and microangiopathic hemolytic anemia
- microcirculatory lesions cause mechanicla damage to RBCs -> helmet cells/schistocytes
what can cause aplastic anemia?
- hereditary (Fanconi anemia)
- acquired:
- busulfran and vincristine, chloramphenicol, phenylbutazone
- benzene and insecticides
- cytomegalovirus (CMV), parvovirus and hepatitis
myelophthisic anemia
- infiltration of the BM by expansile lesions (metastatic carcinoma) that DISTORT the bone marrow architecture, and generally depress the production of all three types of formed blood elements
nl platelet count
autoantibodies to ACh receptor
myasthenia gravis
antibodies to Type IV collagen
- Goodpasture sydnrome
waldenstrom macroglobulinemia
- neoplasms of lymphocytoid plasma cells that make monocolonal IgM
- hypergammaglobulinemia -> hyperviscosity of blood
- tumor causes weakness, weightloss, bone pain, hepatosplenomegaly, and lymphadenopathy
what is a marker of extremely primitive cells of lymphocyte lineages?
- terminal deoxynucleotidyl transferase
- active when VDJ rearrangement of the HC variable domain genes is occuring
- responsible for mediating N-nucleotide addition to the junctions of the gene segments
what are lacunar cells specfic for?
- nodular sclerosing variant of Hodgkin disease
- should also see broad collagen bands on biopsy
- the actual tumor cell in this lesion is the lacunar cell
- the classic Reed sternberg cell is difficult to find