5.18 Heme/onc I

Front 1

1. What is a reticulocyte?

2. What are the locations of the mast cell and the basophil?

3. What is the function/location of the Gp1b receptor?

Back 1

1. an immature RBC

2. mast cell: tissues; basophil: blood

3. binds platelets to damaged endothelium via vWF

Front 2

1. What are the three main blood cell precursors after the pluripotent proginator?

2. Compare the nucleus of the basophil with that of the neutrophil?

Back 2

1. myeloid, lymphoid, erythroid

2. neutrophi: multi-lobed; basophil: bilobed

Front 3

1. What is the difference between poikilocytosis and anisocytosis?

2. What is the difference between a macrophage and a monocyte?

3. Multiple Myeloma is a neoplasm of what cell type?

Back 3

1. aniSo=unequal size; poikilo: unequal shapes

2. monocytes: blood; macrophage: tissue

3. plasma cells

Front 4

1. What do basophil and mast cell granules contain?

2. What cells do mast cells summon? What is the chemotactic factor? Why do they do this?

3. Which lymphocyte is responsible for the humoral response and the cellular response?

Back 4

1. histamine, heparin

2. Eosinophilic chemotactic factors summun eosinophils; eosinophils dampen the immune response w/ histaminase

3. B: humoral; T: cellular

Front 5

1. Hypersegmented PMN are seen in what condition(s)?

2. What are the functions of the eosinophil? (2)

3. What chemotactic factor recruits macrophages? From what/where is it secreted?

Back 5

1. B12 or folate deficiency

2. parasite/helminth protection and asthma

3. Th cells secrete INF-g to recruit macrophages

Front 6

1. What is the difference between primary hemostasis and secondary hemostasis?

2. What types of platelet granules are there and what are their contents? (4)

3. What is the function of dendtritic cells?

Back 6

1. primary is platelets forming initial clot; secondary is clotting factors forming fibrin to stabilize clot

2. Dense: ADP/Ca+2; Light: vWF, fibrinogen

3. professional APCs

Front 7

1. What is the proginator of the plasma cell?

2. What is the function of Protein C?

2. What is the function of the GpIIb/IIIa receptor

Back 7

1. the B cell

2. Inactivates Va/VIIIa

3. binds platelets to other platelets

Front 8

1. Draw the coagulation cascade

2. What is a Factor V Leiden mutation and its consequences?

3. Which clotting pathway is more important?

Back 8

1. SEE SHEET

2. Makes Va resistant to inactivation by aProtein C (thrombotic disease)

3. Extrinsic

Front 9

1. PT/PTT are associated with which pathways?

2. What is the initial stimulus for extrinsic/intrinsic pathways?

3. What is von Willebrand's disease?

Back 9

1. PT: extrinsic; PTT: intrinsic

2. extrinsic: tissue damage; intrinsic: vessel damage

3. Defective vWF prevents effective platelet binding to damaged endothelium

Front 10

1. What is the function of vWF? (2)

2. How is Protein C activated?

3. What is Bernard/Soulier's disease?

4. What is the function of XIIIa?

Back 10

1. intermediate molecule between damaged tissue (exposed collagen) and Gp1b receptor on platelets; protects/carries VIII

2. Activated by Protein S (thrombomodulin)

3. Defective Gp1b prevents effective platelet binding to damaged endothelium

4. cross-links fibrin clot

Front 11

1. What are the clotting factors produced by the liver?

2. Draw the convergence of the coagulation and kinin pathways?

Back 11

1. 2, 7, 9, 10, C, S

2. SEE SHEET

Front 12

1. What is the MOA of heparin? What clotting factors are effected?

2. What is the MOA of warfarin? What clotting factors are effected? (HINT: WHAT ENZYME?)

Back 12

1. Heparin binds/activated AT-III (2a, 10a)

2. Warfarin inhibits epoxide reductase, enzyme required for synthesis of liver clotting factors (2, 7, 9, 10)

Front 13

1. What is the function of AT-III (specific factors)?

2. Compare the origins/functions of PGI2 and TXA2?

Back 13

1. cleaves IIa and Xa

2. PGI2: endothelium/anti-coagulative; TXA2: platelet/coagulative

Front 14

1. What is the cause of a schistocyte?

2. In what pathology is a "bite cell" seen?

3. What is the function of the Coombs test?

Back 14

1. hemolytic anemia

2. G6PD deficiency

3. differentiates between immune and non-immune RBC hemolysis

Front 15

1. In what pathology is a "target cell" seen?

2. In what pathology is a "tear drop cell" seen?

3. Describe the sizes of RBCs in thalassemias?

Back 15

1. thalassemia

2. myelofibrosis ("crying cause its losing all its friends!")

3. RBC are microcytic

Front 16

1. What is the function of haptoglobin and how is it affected in hemolysis?

2. What is the cause of the iron deficiency in anemia of chronic disease?

Back 16

1. haptoglobin binds free Hgb; decreased levels in hemolysis (↑binding)

2. Cells are holding on to their iron

Front 17

1. What is a porphyria?

2. Why is TIBC decreased in anemia of chronic disease?

Back 17

1. abnormality in the synthesis of heme

2. evolution doesn't want iron in the blood when a pathogen could take it

Front 18

1. Draw the Heme Synthesis pathway and mark the enzymes involved in 3 porphyrias?

2. What is the function of ferritin?

3. What type of hemolysis (intra/extravascular) is Paroxysmal Nocturnal Hematuria?

Back 18

1. SEE SHEET

2. binds extra iron for storage

3. intravascular

Front 19

1. What is the function of transferrin?

Back 19

1. moves iron into the system

Front 20

1. Draw out the G6PD function pathway?

Back 20

↑G6PD → ↑NADPH → ↑glutathione → ↓H2O2

Front 21

1. What is the most common bleeding disorder?

2. What's is Glanzmans Thrombasthenia?

Back 21

1. Von Willebrand disease

2. ↓IIb/IIIa

Front 22

1. What is the etiology of ITP?

2. Which clotting factors does PT and PTT measure?

Back 22

1. PLT Ab to IIb/IIIa

2. PT: 7 PTT: 8/9

Front 23

1. What is the etiology of TTP?

2. What are the cellular markers of a Reed-Sternberg cell?

Back 23

1. deficiency of vWF cleaving proteases

2. CD15+, CD30+

Front 24

1. What is the general cause for an increase in bleeding time?

2. What are the good prognostic indicators for Hodgkins lymphoma?

Back 24

1. PLT defects

2. ↑Lymphocyes/↓Reed-Sternberg cells

Front 25

1. What is the age distribution of HLs and NHLs?

2. What is the difference between Multiple Myeloma and Waldenstrom's Macroglobinemia?

Back 25

1. HLs: young/old bimodal, NHLs:20-40

2. MM is IgG; WM is IgM

Front 26

1. t(8;14) causes what? Also what oncogene is associated with this?

2. Compare acute and chronic leukemias with respect to the maturity of the cells produced?

Back 26

1. burkitt's lymphoma; c-myc

2. acute: blasts; chronic: cytes

Front 27

1. t(14;18) causes what? Also, what oncogene is associated with this?

2. Compare acute and chronic leukemias with this course?

Back 27

1. follicular lymphoma; bcl-2

2. acute: abrupt/stormy, chronic mild, less severe

Front 28

1. "Starry Sky" pattern on histology is associated with what disease? What is the stary sky referring to?

2. What is histiocytosis X? For bonus, what is the cell marker for this proliferative cell?

Back 28

1. Burkitt's lymphoma; sheets of lymphocytes with interspersed macrophages

2. Clonal proliferation of Langerhans cells: CD1a+

Front 29

1. AML responds pharmacologically to what compound?

2. Which leukemia is associated with a "blast crisis"

Back 29

1. all-trans retinoic acid (vit A)

2. CML

Front 30

1. With CML, the mutation involved in the myeloproliferative disorder is abr-bcl. What is the mutation involved with the other myeloproliferative diseases? (HINT: What ARE the other myeloproliferative diseases?)

Back 30

1. JAK2 mutations

(Polycythemia Vera, Essential Thrombocytosis, Myelofibrosis, CML)

Front 31

1. What is meant by "activated" platelets?

2. Describe the mechanism of HIT?

3. What is the difference in activity between unfractionated and LMWH?

Back 31

1. Degranulation and IIb/IIIa to surface in order to aid in clotting

2. LWMH + PF4 form Ag/Ab complex that activate platelets and form clots

3. Fractionated works on both IIa and Xa; LWMH is selective for Xa

Front 32

1. Why would you administer bivalirudin/lepirudin instead of heparin? How does its MOA differ from that of heparin?

2. Which drug crosses the placenta: heparin or warfarin?

3. Why do sickle-cell patients have auto-splenectomy?

Back 32

1. patient sensitive to HIT; binds directs to IIa to inactivate (no AT-III intermediary)

2. be "wary" of WARFarin

3. sludging of sickle cells causes vascular block in spleen

Front 33

1. What lab values monitor for Heparin and for Warfarin?

2. Compare the duration of action of heparin and warfarin?

3. What conditions exacerbate sickle cell?

Back 33

1. EX-PresidenT went to WAR
(IN-PTT-Hep)

2. Heparin: hours; Warfarin: until new factors are made

3. dehydration and low O2

Front 34

1. What are the antidotes for Heparin and Warfarin toxicities?

2. What does plasminogen do?

3. What populations do α and β thalassemias effect? (2)

Back 34

1. Heparin: protamine; Warfarin: Vit K + FFP

2. cleaves thrombin and fibrin clots

3. α: asian/african β:mediterranean

Front 35

1. What conditions have a "crew cut" appearance on X-ray?

2. What is the difference between β-thalassemia major and minor?

3. Why are newborns with sickle cell initially asymptomatic?

Back 35

1. thalassemias, sickle cell

2. major: homozygote; minor: heterozygote

3. HbF synthesis, not HbS

Front 36

1. Which bacteria is associated with a positive cold-agglutinin test?

2. What Ig is cold agglutinin and warm agglutinin?

Back 36

1. Mycoplasma pneumoniae

2. Warm: IgG, Cold: IgM

Front 37

1. What is the difference between Direct Coombs and indirect Coombs?

2. What is the cause of erythroblastosis fetalis?

Back 37

1. Direct: Ig added to RBC and watch for agglutinatio; Indirect: RBCs added to serum and watch for agglutination

2. Rh+ fetus with maternal anti-Rh+

Front 38

1. What is the defect in hereditary spherocytosis? What does it cause?

Back 38

1. ankyrin/spectrin or band 3.1 causes round RBCs; causes round RBC

Front 39

1. What is the difference between Extra/Intravascular hemolysis? Can you give two examples of intravascular hemolysis?

Back 39

1. Intra in the veins; microangiopathic anemia AND paroxysmal nocturnal hemoglobinuria