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Hemeonc

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Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
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This is a study tool. The 3 wrong answers for each question are randomly chosen from answers to other questions. So, you might find at times the answers obvious, but you will see it re-enforces your understanding as you take the test each time.

1. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
They bind vWF via GpIb
VWF carries/protects factor VIII
DIC - TTP/HUS - traumatic hemolysis
Her next Rh+ fetus

2. What are the extrinsic hemolytic normocytic anemias?
Hereditary elloptocytosis
Sheets of lymphocytes interspersed with macs
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
...

3. What is the pathogenesis of aplastic anemia with kidney disease
CD15 and CD30 pos
Decrease EPO
B cells
Aplastic anemia - pancytopenia

4. What do auer rods stain with
All trans retinoic acid
Degrades fibrin mesh and converts C3 to C3a
ADP and Ca
Peroxidase

5. What is the therapy for the M3 variant?
Epoxide reductase - warfarin inhibits
All trans retinoic acid
Tissue factor converst the VII and VIIa
Fe def - thal - pb poison - sideroblastic anemia

6. Why can newborns with sickle cell be asymptomatic
Inc HbF and dec HbS
Exposed collagen upon endothelial damage
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
PGI2 - NO inc blood flow - dec platelet aggregation

7. What is factor V leidin?

8. Teardrop cell
Indirect coombs - agglutinate if serum anti RBC surface Ig
Extrinsic - I - II - V - VII and X
Fibrinogen
Bone marrow infiltration - myelofibrosis

9. What role does antithrombin play?
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Incactivates II - VII - IX - X - XI - XII
Down - up - down
CNS and testis

10. RBCs damagaed from passing through obstructed - narrowed vessel lumina
Liver disease - abetalipoproteinemia - acntho = spiny
Splicing sites and promotor sequences
MAHA
Schistocytes and inc LDH

11. What do the platelets bind? What is the step called
Exposed collagen upon endothelial damage
Extravascular hemolysis
Gp1b
They bind vWF via GpIb

12. Drug that inhibits COX and therefore TXA2 synthesis
Protein S - cleaves and invactivates Va and VIIIa
Imatinib - anti bcr abl
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Aspirin

13. What does LEAD stand for in lead poisoning?
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -
Tissue factor converst the VII and VIIa
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Inappropriate absolute with inc RBCs and EPO

14. What do they express on their surface?
Ringed sideronblasts with iron laden mitochondria
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
MHC II and Fc receptors

15. What do you see in peripheral smear in a pt with G6PD?
Bite cells and Heinz bodies
A antigen and B antibodies
CLL (SLL without the peripheral lymphocytosis
Pyruvate kinase def - extravascular

16. t(15;17)
Spleen
Protoporphyrin
Abciximab
M3 AML (acute promyelocytic leukemia)

17. Where to T cells arise and mature - and migrate to...
Bone marrow - thymus - blood (80% of circulating lymphos are T)
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Dec synthesis of factors 1972 - protein C/S
DIC - TTP/HUS - traumatic hemolysis

18. what proteins can be defective in HS?
EBV
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Ankryin - band 4.1 or spectrin
ATIII def

19. What do you see on peripheral smear with sideroblastic anemia
Dendritic cells?
Ringed sideronblasts with iron laden mitochondria
Inc
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

20. What is the general pathology of a macrocytic anemia?
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

21. Is HS extravascular or intravascular?
Extravascular
Intravascular
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

22. What is hemophiliia A
M3 AML (acute promyelocytic leukemia)
Up - down - nl
Deficiency in factor VIII
CD15 and CD30 pos

23. t(8;14) c - myc gene
Burkitt lymphoma
Extravascular hemolysis
Produce antibody - lots of RER and golgi
B12 def

24. What virus can cause an aplastic crisis in pts with HS?
Up - down - up
Plummer - vinson syndrome
Parvovirus
Fibrinogen bind GpIIb/IIIa and links platelts

25. What are the main associations with multiple myeloma?
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Sideroblastic anemia
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

26. What are the variants of the RS cells
120 days
Lacunar in nodular sclerosing variant
ADP and Ca
Follicular lymphoma - indolent course

27. What percentage of WBCs are monocytes?
2 to 10
Alpha thal - asian and african american
Abciximab
Helps platelts adhere to endothelium

28. What does the large SA:volume ratio in RBCs help facilitate?
Histamine - heparin - and eosinohil chemotactic factors
Thrombotic thrombocytopenic purpura
Easy gas exchange
No platelet clumping

29. Where do B cells arise from - mature - and migrate to...
Uroporphyrinogen decarboxylase
Spleen
Myelofibrosis (marow is crying being its fibrosed'
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

30. universal donor
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
No antigen - both antibodies
Positive osmootic fragility test and splenectomy
DIC - TTP/HUS - traumatic hemolysis

31. What is the most common nonhodgkin lymphoma
VWF and fibrinogen
Signs and sx of aplastic anemia
Diffuse Large b cell lymphoma
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -

32. What virus is associated with Burkitt lymphoma
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Inc HbF and dec HbS
EBV
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

33. When is the peak incidence for nonHod lymphoma
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
20 to 40
Bite cells and Heinz bodies
Tissue factor converst the VII and VIIa

34. hypocellular bone marrown tih fatty infiltration
DIC
Burkitt lymphoma
Protein C or S deficiency
Aplastic anemia - pancytopenia

35. Serum iron - transferrin - ferritin lab values for hemochromatosis
Anemia of chronic disease - Aplastic anemia - kidney disease
Up - down - up
Helminth infections major basic protein
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

36. What does the blood smear show in glanzmann's?
Deficiency in factor VIII
Anti Rh
No platelet clumping
No antigen - both antibodies

37. What is the characteristic finding for MAHA on peripheral smear?
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -
Schistocytes - helmet cells
Paraprotein spike - monoclonal protein
Ringed sideronblasts with iron laden mitochondria

38. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
EBV
Protein C or S deficiency
Howell Jolly bodies
Megakaryocytes

39. What is the pathogenesis of TTP?
Kids= exposure to lead paint - adults = battery - ammunition factory
90% anearobically from glucose to lactate - 10% from HMP shunt
Uroporphyrin (tea colored urine)
Inc large vWF multimers - inc platelet aggregation and thrombosis

40. What is their role?
Prothrombin gene mutation
Eleveated PT - PTT
Inducers of primary antibody response
Nl PT - elevated PTT - intrinsic pathway defect

41. Where do you see hypersegmented polys?
Howell - Jolly bodies - hypo/asplenia
Positive osmootic fragility test and splenectomy
Sheets of lymphocytes interspersed with macs
B12/folate def

42. What is the philadelphia chromosome
Beta 4
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
M3 AML (acute promyelocytic leukemia)
T(9;22) bcr abl

43. tennis rackets on EM
Howell - Jolly bodies - hypo/asplenia
T(9;22) bcr abl
Birbeck granules
Hb Barts - gamma4 in defect in all 4 alpha genes

44. What is the mutation in HbS
Valine for glutamate
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Protoporphyrin (blood)

45. Deficiency in GpIIb/IIIa

46. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
ATIII def
CLL (SLL without the peripheral lymphocytosis
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Beta thal

47. What is the ddx for aplastic anemia

48. After an injury - What does vWF bind to begin platelet plug formation?
Exposed collagen upon endothelial damage
EBV
Easy gas exchange
DIC

49. How does the therapy for M3 vairant work?
Signs and sx of aplastic anemia
Idiopathic thrombocytopenia (ITP)
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -
Induces differentiation of myeloblasts

50. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Low in CML
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Uroporphyrinogen decarboxylase
Off center nuclues - clock face chromatin