Step 1 Heme/onc

Front 1

What is the syndrome and what is it associated with: Migratory thrombophlbitis due to hypercoaguability.

Back 1

Trousseaus syndrome associated with visceral malignancy occurs because adenocarcinoma produce a thromboplastin like substance that is capable of causing chrnoic intravascular coagulations that are disseminated and tend to migrate.

Front 2

what does GMCSF stimulate and decrease

Back 2

Stimulates myeloblast line decreases mast cells

Front 3

What is anisocytosis? and Poikilocytosis

Back 3

anis - different sizes
Poik - different shapes

Front 4

In platelets what are contained in the dense granules, and in the alpha granules

Back 4

Dense = adp, ca, 5ht
alpha - vwf, fibrinogen, pdgf,pf

Front 5

platelet lifespan?

Back 5

8-10 d

Front 6

1/3 of the platelet pool is stored in the __________

Back 6

Spleen

Front 7

what are the VWF and fibrinogen receptors?

Back 7

vWF - Gp1b
Fibrinogen - GpIIb/IIIa

Front 8

What cell type has a long life in the tissues?

Back 8

Macrophages

Front 9

What cells are highly phagocytic for ag-ab complexes?

Back 9

Eos

Front 10

What do the dense basophilic granules contain?

Back 10

Histamine, heparin, leukotrienes (LTD4)

Front 11

What is the syndrome and what is it associated with: Migratory thrombophlbitis due to hypercoaguability.

Back 11

Trousseaus syndrome associated with visceral malignancy occurs because adenocarcinoma produce a thromboplastin like substance that is capable of causing chrnoic intravascular coagulations that are disseminated and tend to migrate.

Front 12

what does GMCSF stimulate and decrease

Back 12

Stimulates myeloblast line decreases mast cells

Front 13

What is anisocytosis? and Poikilocytosis

Back 13

anis - different sizes
Poik - different shapes

Front 14

In platelets what are contained in the dense granules, and in the alpha granules

Back 14

Dense = adp, ca, 5ht
alpha - vwf, fibrinogen, pdgf,pf

Front 15

platelet lifespan?

Back 15

8-10 d

Front 16

1/3 of the platelet pool is stored in the __________

Back 16

Spleen

Front 17

what are the VWF and fibrinogen receptors?

Back 17

vWF - Gp1b
Fibrinogen - GpIIb/IIIa

Front 18

What cell type has a long life in the tissues?

Back 18

Macrophages

Front 19

What cells are highly phagocytic for ag-ab complexes?

Back 19

Eos

Front 20

What do the dense basophilic granules contain?

Back 20

Histamine, heparin, leukotrienes (LTD4)

Front 21

What are three functions of bradykinin

Back 21

Vasodilation, inc permeability, and increased pain

Front 22

What does tPA catalyze

Back 22

Plasminogen --> plasmi

Front 23

What lab tests is used to determine the presence of clots (

Back 23

D-dimer note this is not specific but if it isn't present you can be pretty sure there isn't a clot/embolism

Front 24

Antithrombin inhibits what?

Back 24

2 (thrombin),7,9,10,11,12

Front 25

what does factor 13 do?

Back 25

cross links via disulfide bonds the fibrin to stabilize the clot

Front 26

what are the two functions of ADP in platelet plug formation

Back 26

1) helps platelets adhere to endo
2) increases expression of gpIIb/IIIa at platelet surface

Front 27

What activates protein C

Back 27

Thrombomodulin (on vasc endothelial cells)

Front 28

What is the function of TxA2 produced by platelets

Back 28

It promotes the release reaction change in shape, and aggregation

**Aspirin inhibits this

Front 29

What causes acanthocytes (spur)

Back 29

Liver disease, Abetalipoproteinemia

Front 30

What causes Burr cells

Back 30

These are cells with uniform projections (unlike acanthos) caused by uremia, pyruv kindase def, trauma (long distance running)

Front 31

What are the causes of basophilic stippling

Back 31

Thalessemia,, anemia of chronic disease, iron def, lead poisoning

Front 32

What causes elliptocytes

Back 32

AD, defect in 4.1 or glycophorin C usually asymptomatic, get squeezed in the caps and can't puff back up

Front 33

What causes ringed sideroblasts

Back 33

Sideroblastic anemia (ala synthase def)

Front 34

What are cuases of schistocytes

Back 34

DIC,TTP/HUS, traumatic hemolysis

Front 35

What are some signs and symtoms of spherocytosis

Back 35

Increases MCHC is pathognomonic, inc risk of black gallstones and b19 induced aplastic anemia

Front 36

What are causes of target cells

Back 36

Hbc, asplenia, liverdisease, thalassemia, herditary LCAT deficiency

Front 37

What is the process that creates Heinz bodies,

Back 37

Oxidation of iron from ferrous (2+) to ferric (3+) leads to denatured Hb precipitation and damage to the RBC membrane....leads to pite cells...see with alpha thal and G6PD

Front 38

What things can precip G6PD issues?

Back 38

Dapsone, primaquine, cholarquine, fava, DKA, sulfa, nitro

Front 39

What is the triad of Plummer Vinson? Associated condition?

Back 39

Anemia, esophageal web(dysphagia), glossitis. Associated with esohpageal SCCC

Front 40

What type of mutation of affects alpha thals?

Back 40

Deletion

Front 41

Where do you see hemoglobing bart

Back 41

Gamma 4 deletion of all 4 alpha genes, can cause hydrops fetalis

Front 42

Where do you see HbH

Back 42

B4, deletion of three alpha genes, see get extravasc hemo, clinically similar to B thalassemia major

Front 43

What types of muts typically cause B thal

Back 43

Muts that affect the transcription and translation of B chain

Front 44

What lab test confirms B thal

Back 44

Increased HbA2 on electrophoresis(alpha2delta2) and an increase in

Front 45

What are the signs and symptoms of B thal major? tx?

Back 45

Blood transfusion (risk of hemochromatosis without chelation thearpy), marrow expansion (crew cut or chipmunk face)

Front 46

HbS/B thal heterozygote presentation

Back 46

Mild to moderate sickle cell disease depending on the amount of B globin produced

Front 47

Sideroblastic - whats the inheritance? Treatment?

Back 47

XLR, B6

Front 48

What are some signs and sx of lead poisoning, how do you treatit

Back 48

Lead lines on gingavae and epiphysis, Encephalopthy, basophilic stippling, sideroblastic anemia, abdominal cholic, wrist and phot drop.
Tx: Dimercaprol and EDTA, succimer for kids

Front 49

Whats the def: Megaloblastic anemia Increased Methylmalonyl CoA and inc homocysteine

Back 49

B12 def

Front 50

Where are B12 and folate absorbed

Back 50

ileum, jejunum

Front 51

What are some causes of Nonmegaloblastic macrocytic anemais

Back 51

Liver dz, alcoholism, reticulocytosis (retics are bigger than RBCs), metabolic disorder (orotic aciduria) or congenital def in purine pyrimadine metab pathways, and drugs (hydrozyurea, AZT, 5FU)

Front 52

What are the findings of intravascular hemolysis

Back 52

Dec haptoglobing, Inc LDH, hemogloin in urine

Front 53

What are the findings of extravascular hemolysis

Back 53

Macrophage in spleen clears the RBC, see inc LDH plus INC UCB (macro converts Hb to bili but liver get overwhelmed and can't conjugate fast enough, this causes jaundice (hereditray sphero, G6PD, sickle cell anemia)

Front 54

Lab results of anemia of chronic diseae

Back 54

TIBC dec, Ferritin inc, Iron, dec

Both hepcidin and ferritin are acute phase proteins

** NOTE AGD can become a microcytic anemia given enough time

Front 55

Causes of aplastic anemia

Back 55

1) radiation, and drugs (chloramphenicol, alkylating agents)
2) viruses (Parvo, ebv, hiv, hcv)
3) Fanconi's anemia (defect in DNA repair)
4) idiopathic (immune mediated, primary stem cell defect) may follow acute hepatitis

Front 56

What are the sx of aplastic anemai

Back 56

Fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection

Front 57

what are the mutations in Spherocytosis

Back 57

ankyrin, 4.1, spectrin

Front 58

What type of hemolysis is G6Pd, what are symptoms

Back 58

ONLY INTRAVASC, back pain followed by hemolysis a few days later

Front 59

What are some complications in sicklecell disease

Back 59

Aplastic crisis (B19), autosplenectomy, occurs in early childhood, salmonella osteomyelitis, pain crisis, renal pap necrosis (due to low o2 in the papilla) and microhematuria (due to medullary infarcts)

Front 60

whats the mutation in HbC dz

Back 60

Glutamic acid to lysine (- to a +) migrates the least far on a gel

Front 61

Whats the deficiency in paroxyysmal nocturnal hemoglobinuria

Back 61

Gpi anchor and Decay accelrating factor on the RBC membrane (these prevent activation of the complement system against self RBCs) see hemosiderin in the urine.

Front 62

What causes cyanosis with a normal pO2

Back 62

Methemoglobinemia (MetHb absorbs the two light wavelengths they use to test po2.

Front 63

what is associated with Warm agglutinin anemia, what type of hemolysis, and what isotype

Back 63

SLE, CLL, or drugs (methyldopa), Extravascular, IgG

Front 64

what is associated with cold agglutinin anemia, what type of hemolysis, and what isotype

Back 64

CLL< mycoplasma, EBV, infections, intravascular, igM

Front 65

what does a direct coombs detect

Back 65

abs on a cell

* the test uses anti Ig ab added to patients rbcs, cells will agglutinate if they are coated with Ig

Front 66

What does an indirect coombs detect

Back 66

Free floating abs

* Normal rbs are added to patients SERUM, agglut if serum has anti rbc surface - used for crossmatching

Front 67

What are causes of microagniopathic anemia

Back 67

DIC,TTP-HUS, SLe, and malignant hypertension
*see schistocytes

Front 68

What two infections cause destruction of RBCs (hemolytic anemia)

Back 68

Malaria, babesia

Front 69

What are the lab values in hemochromatosis

Back 69

Iron ^ ferritin ^ TIBC dec %saturatino of transferring ^

Front 70

What are the lab values in pregnancy/OCP use

Back 70

Serum iron same, TIBC inc, Ferritin, no change, % transferrin dec.

Front 71

lead poisoning

Back 71

Can't use iron (can't make heme due to enzyme inhibition) Iron inc, TIBC dec, ferritin no change, % transferrin inc

Front 72

What enzymes are effected by lead poisoning?

Back 72

Ferrochelatase and ALA dehydratase.
*Kids get it from lead paint --> mental deterioration
*Adults get it from environmental exposure (batter/ammo factory) --> headache mem loss demyelination

*** General - microcytic anemia, GI and kidney disease.

Front 73

What is the accumulates in the blood or urine in Lead poisoning, AIP, PCT?

Back 73

1. Protoporphyrin (blood)
2. Porphobilinogen, s-ala, uroporphyrin (Urine)
3. Uroporphyrin (urine)

Front 74

AIP what is the enzyme def and sx

Back 74

this is AD!! def is in porphobilinogen deaminase. Symptoms - painful abdomen, red wine colored urine, polyneuropathy, psychological disturbanes, precipitated by drugs (phenobarb, thiopental)

Front 75

How do you treat AIP

Back 75

Glucose and heme (both inhibit ALA synthase)

Front 76

HWhat is the enzyme def in PCT and sx

Back 76

Uroporphyrinogen decarboxylase, blistering cutaneous photosensitivity, hypertrichosis, most common porphyria

Front 77

What is the first step in porhyrin syn

Back 77

Glycine + Succinyl Coa --> ALA (uses B6 as a cofactor) thats why you treat sideroblastic with B6

Front 78

Whats the disorder: macrohemorrhage inc PTT

Back 78

Hemo A (8) or B (9)
* can treat factor 8 deficiency with ddavp

Front 79

What's the disorder: Increased PT increased PTT

Back 79

Vit K def

Front 80

What's the disorder: dec PC increased BT labs otherwise normal

Back 80

Bernard soulier, dec GP1B defect in platelet adhesion to collagen see giant platelets

Front 81

What's the disorder: normal PC increased BT, blood smear shows NO platelet clummping

Back 81

Defect in plug formation GLANZMAN THROMBASTHENIA dec GpIIb/GPIIIa,

Front 82

What's the disorder:: Dec PC inc BT, increase in megakaryocytes in BM

Back 82

Dec platelet survival, anti GpIIb/IIIa get peripheral platelet destruction

Front 83

What's the disorder: dec PC inc BT, inc LDH, see large multimers, schistocytes

Back 83

dec platelet survival, def of adamts 13 (vWF metalloprotease) dec degradation of multipers, inc plate agg and thrombosis. Pentad of neuro (stoke like) renal, fever, thrombocytopenia, and microangiopathic hemolitc anemia.

Front 84

What are the s/sx of heparin induced thrombocytopenia

Back 84

* occurs 5-14 days after hep is started
Heparin binds to platelet ag (factor 4) an autoantibody forms against hep/factor4 complex that activates platelets leading to their clearance resulting in a thrombocytopenic, hypercoagulable state.

Front 85

What's the disorder: normal BC, inc BT, normal PT and inc/normal PTT

Back 85

vWF disease (AD), carries factor 8 so you can see changes in PTT
** treat with ddavp (releases stored vWF from the endothelium)

Front 86

What's the disorder: Dec PC, inc PT, Inc PT, Inc PTT

Back 86

DIC

Front 87

What are the causes of DIC

Back 87

GN sepsis, Trauma, Obstetric comps, acute panc, malignancy, nephrotic syndrome, transfusion, shistocytes, inc ddimer, dec 5 and 8

Front 88

What's the disorder: mutant factor five that cannot be degraded by prot c

Back 88

Factor V leiden...most common inherited hypercoaguability

Front 89

What gene mutation results in the 3 prime untranslated region mutation and is associated with venous clots

Back 89

Prothrombin gene mut

Front 90

What increases the risk of thrombotic skin nerosis with hemorrhage following the administration of warfarin

Back 90

Protein C or S deficiency Inability to inactivate factors 5 and 8

Front 91

What are the lab results in leukemoid reaction

Back 91

Inc WBC count with left shift, (80%) bands, inc leuck alk phos, usually do to infection

Front 92

What are some features of Hodgkins

Back 92

RS cell (CD30+, CD 15+), localized, single group of nodes, extranodal, rare; contiguous spread (stage is strongest predictor of prognosis), constitutional sx, low grade fever nigh sweat weight loss, mediastinal lymphadenopathy, 50% of cases associated with EBV (bimodal dist), good prognosis with high lymphos low RS cells, most are more common in med except nodular sclerosing

Front 93

What are some features of NHL

Back 93

Assoc with HIV, immunosuppression, multiple peripheral nodes, extranodal involvement, noncontiguous spread, Majority involve B (except for T cell origin), fewer constitutional signs/symptoms, Peak incidence for certain subtypes at 20-40 yrs

Front 94

What Hodgkins lymphoma has many lymphos and lacunar cells

Back 94

Nodular sclerosing, lacunar cells look kinda like kilocytes. Women >Men, most common, young adults

Front 95

What type has high RS med lymphos and int prognosis

Back 95

Mixed cellularity

Front 96

Where do you see lymphocyte dom HL (what pop?)

Back 96

<35yo males

Front 97

What Hodgkins lymphoma has the worst prognosis

Back 97

Lymphocyte depleted older males with disseminated disease, assoc w/ HIV

Front 98

Whats the NHL (8:14) what are the symptoms of the sporadic form, cymc gene moves next to heavy ig Chain

Back 98

Burkitts, stary sky appearance, sporadic is in pelvis or abdomen

Front 99

What is the most common adult NHL (CD 19+, CD20+)

Back 99

Diffuse large cell, very aggressive presents as a rapidly enlarging mass anywhere in the body

Front 100

NHL with an 11:14 translocation, CD5+, CD23 -

Back 100

Mantle cell, poor prog, incurable, overexpression of cyclin D

Front 101

NHL with a 12:18 translocation and expof Bcl2, CD 10+

Back 101

Follicular, hard to cure, bcl usually inhibits apoptosis

Front 102

What lymphoma is cause by HTLV-1

Back 102

Adult T cell lymphoma, adults present with cutaneous lesions, especially affects pops in Japan, W african, and the caribbean. Aggressive. Hypercalcemia

Front 103

Mycosis fungoides/sezary syndrome - sighns symptoms

Back 103

Presents with cutaneous patches or nodules, CD4+

Front 104

Most common primary bone tumor in the elderly?

Back 104

Multiple myeloma

Front 105

What does the multiple myeloma CRAB acronym stand for

Back 105

C = Hypercalcemia
R - Renal insufficiency
A= Anemia
B= bone lesions/back pain

Front 106

What occurs in waldenstroms macroglobulinemia

Back 106

M spike is IgM usually see hyperviscosity symptoms no lytic bone lesions

Front 107

MGUS

Back 107

Monoclonal plasma cell expansion without the sx of multiple myeloma, without bence jones.

Front 108

What happens to leukocytes in BM and circulation

Back 108

They go go up or down but usually lead to bone marrow failure and anemia, infections, hemorrhages (RBCS, mature WBC, platelet dec) and leukemic cell infiltrates in the liver, spleen and LNs are possible.

Front 109

what is the age rage in ALL

Back 109

0-14, assoc with downs

Front 110

What is the cellular markers in ALL

Back 110

TdT + marker of pre t and B cells and CALLA (cd10) for the B cell ones

Front 111

What translocation that signifies better prognosis in ALL...what percent of blasts should you see

Back 111

12:21, >30

Front 112

Whats the age rage for SLL/CLL (SLL =small lymphocytic lymphoma)

Back 112

>60

Front 113

What do you see in the peripheral blood smear

Back 113

Smudge cells, warm antibody AI hemolytic anemia, hypogammaglobinemia

Front 114

What are the markers for CLL

Back 114

T cell = CD5+ CD 23+

Front 115

What is the age range for hairy cell leukemia

Back 115

Elderly and adults

Front 116

How does haircell present, what are the labs

Back 116

Present with pancytopenia and splenomegaly, Cells have filamentous hair like projections and stain TRAP (tartrate) positive.

Front 117

How do you treat hairy cell

Back 117

2Chloroxyadenosine

Front 118

What is the age range for AML

Back 118

15-59

Front 119

What are the findings in AML

Back 119

Auer rods inc circ myeloblast on peripheral smear

Front 120

What is the M3 AML

Back 120

15;17 translocation and can treat with vitamin A which will induce differentiation of the myeloblasts
DIC risk

Front 121

What is the age range for CML

Back 121

35-50

Front 122

What is the defining chromosomal translocation and med for CML

Back 122

9:22 (philidelphia) and intimab..See inc neutros, metamyelos, basophils, splenomeg, may accelerate and transform into AML or ALL (blast crisis)
*** ALK PHOS IS VERY LOW (unlike leukemoid reaction)

Front 123

What is mastocytosis

Back 123

Inc mast cells --> inc histamine --> inc gastic acid (this will inactivate pancreatic enzymes) --> diarrhea --> N/V abn cramp, ulcer, syncope flushing, tachy, bronchospasm urticaria

Front 124

Whats the transloation in ewings sarcoma

Back 124

11;22

Front 125

What is langerhands histiocytosis

Back 125

Prolif disorder of langerhans cells. Defective cells express s-100 and CD1a birbeck granules (tennis rackets) are characteristic.
** occurs in kids and young adults can see a rash.

Front 126

What causes inc RBCs, Wbcs, and platelets, whats the Mutation and associations, RBC mass increases

Back 126

Polycythemia vera, JAK2 mutation, assoc w/ RCC, Wilms tumor, cyst, HCC, hydronephrosis

Front 127

What causes increased Platelets specific for megakaryocytes

Back 127

Essential thrombocytopenia, JAK 2 mut in 30-50%

Front 128

What causes decreased RBCs and variable WBC and platelet, and tear cells

Back 128

Myelofibrosis...Most common cause of splenomegaly >50 Can see splenic infacrct and a L pleural effusion

Front 129

What causes Dec RBCs inc WBCs, and Inc Platelets, whats the gene

Back 129

CML Philedeplphia chromo

Front 130

What are some causes of appropriate absolute polycythemia

Back 130

Lung disease, congenital heart disease, high altitude

Front 131

What are some hyperviscosity symptoms

Back 131

Hepatosplenomegaly, burning pain in hands (see this is ET too) uddy face, thrombosis, CNS involvement, inc histamine (itchiness after bathing ), gout,

Front 132

What conditions cause increases in leuckocyte alanine phophatase

Back 132

1) polycythemia vera, esstntial thrombocytopenia, primary myelofibrosis and leukemoid rxn

Front 133

What conditions cause a decrease in Leuckocyte alanie phospohatase

Back 133

CML, PNH, AML