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Hemeonc

Instructions:

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. What does LEAD stand for in lead poisoning?
Blood
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 -
Varying sizes
Howell Jolly bodies

2. How are the nucleus and the cytoplasm characterized for lymphocytes
IFN gama
Parvovirus
Round densly staining nucleus with a small amount of pale cytoplasm
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

3. Which maternal antibodies cross the placenta - anti A/B or anti Rh
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
...
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Anti Rh

4. What are the labs and tx for HS?
TXA2 - dec blood flow - inc platelet aggregation
Dec
VIII
Positive osmootic fragility test and splenectomy

5. What is the presenting scenario for TTP?
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Parvovirus
Nodular sclerosing
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

6. Schisotcyte - helmet cell
Langerhans cell histiocytosis
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
DIC - TTP/HUS - traumatic hemolysis
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

7. Which drugs can cause macrocytic anemia?
5- FU - AZT - hydroxyurea
Lung disease - congenital heart diseaes - and high altitude
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
AB - no antibodies

8. What is hemophiliia A
Bleeding - malnutrition/absorption - inc demand pregs
Inc HbA2 on electrophoresis
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Deficiency in factor VIII

9. Blood type B
Myelofibrosis (marow is crying being its fibrosed'
Ferrocheletase and ALA dehydrogenase
B antigena and A antibodes
Down - down - up

10. Target cell

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11. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Paroxysmal nocturnal hemoglobinuria
Extrinsic - I - II - V - VII and X
Rhogam - Rh antigen immunoglobulin
Blasts > 5%

12. inc HbF on electrophoresis
5- FU - AZT - hydroxyurea
No lytic bone lesions in WM
90% anearobically from glucose to lactate - 10% from HMP shunt
Both alpha and beta thal

13. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Factor V Leiden
CLL (SLL without the peripheral lymphocytosis
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Multiple myeloma

14. Which pathway and factorrs are tested by the PT coag test
Extrinsic - I - II - V - VII and X
Intrinsic - all factors except - VII - XIII
CML to AML or All
Myelofibrosis (marow is crying being its fibrosed'

15. Bone pain plus anemia
Hereditary elloptocytosis
Extrinsic - I - II - V - VII and X
Multiple myeloma
No

16. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Bite cells and Heinz bodies
...
B12 def
MGUS - monoclonal gammopathy of undetermined significance

17. What is the tx for vWD
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Birbeck granules
Signs and sx of aplastic anemia

18. What is the result of thrombocytopenia or platelet dysfunction?
Petechiae
Protoporphyrin
Plummer - vinson syndrome
Aspirin

19. Elderly - mature b cell tumor with filamentous - hairlike projections
Inc vasodiltion - inc perm - inc pain
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Hairy cell leukemia
TXA2 - dec blood flow - inc platelet aggregation

20. What is HbH
Beta 4
Lung disease - congenital heart diseaes - and high altitude
Multiple myeloma
Essential thrombocytosis

21. What do you see in peripheral smear in a pt with G6PD?
Bite cells and Heinz bodies
Malaria - Babesia
Inappropriate absolute with inc RBCs and EPO
Multiple myeloma

22. What do labs show in DIC?
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Schistocytes and inc LDH
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Complications of sickle cell anemia

23. What is their role?
Inc RBC - dec O2 sat - inc EPO
Hb Barts - gamma4 in defect in all 4 alpha genes
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Inducers of primary antibody response

24. What do the iron studies show in sideroblastic anemia
Spleen
Hodgkin
Inc serum iron - normal TIBC - inc ferratin
SLE - CLL - alpha methyldopa

25. What are the azuraphilic granules in PMNs
Sideroblastic anemia
B antigena and A antibodes
Birbeck granules
Lysosomes

26. Deficiency in vWF

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27. What do platelets release necessary for coagulation cascaed?
Bernard soulier
ADP and Ca
Exposed collagen upon endothelial damage
Dendritic cells?

28. What is the tx for sickle cell
Hydroxyurea - bone marrow transplant
Cromolyn sodium
Rhogam - Rh antigen immunoglobulin
Protein C or S deficiency

29. What is appropriate absolute polycythemia
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
ATIII def
Inc RBC - dec O2 sat - inc EPO
Positive osmootic fragility test and splenectomy

30. What is the Ddx for nonhemolytic normocytic anemia
Anemia of chronic disease - Aplastic anemia - kidney disease
Multiple myeloma
Deficiency in factor VIII
Mantle cell - older males

31. Deficiency in GpIb
Anemia of chronic disease - Aplastic anemia - kidney disease
B12 in fatty acid pathways leads to subacute combined degeneration
MHC II and Fc receptors
Bernard soulier

32. Megakaryocytosis
Eleveated PT - PTT
Essential thrombocytosis
Blistering cutaneous photosens - most common porphyria
HbSS

33. Deficiency in GpIIb/IIIa

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34. What are the pro aggregation factors?
Protoporphyrin (blood)
TXA2 - dec blood flow - inc platelet aggregation
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Hb Barts - gamma4 in defect in all 4 alpha genes

35. What virus is associated with Burkitt lymphoma
EBV
Sideroblastic anemia
T(12;21)
B6

36. What happens in beta thal major?
Acts to stabilize platelet plug
MAHA
Kids= exposure to lead paint - adults = battery - ammunition factory
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

37. What are the presenting symptoms of acute intermittent porphyria
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Inactivates it
Diffuse Large b cell lymphoma
Plummer - vinson syndrome

38. discrete tumor masses arising from lymph nodes
Porphyrias
Sickle cell anemia
GpIIb/IIIa
Lymphoma

39. What do they express on their surface?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
AML
MHC II and Fc receptors
Aspirin

40. What do the labs show for TTP?
Sickle cell
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Schistocytes and inc LDH

41. iron deficiency anemia - esophageal web - atrophic glossitis
HbSS
Lead - and EtOH
Plummer - vinson syndrome
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

42. hypocellular bone marrown tih fatty infiltration
Fe def - thal - pb poison - sideroblastic anemia
Aplastic anemia - pancytopenia
Fibrinogen bind GpIIb/IIIa and links platelts
No platelet clumping

43. What activates the intrinsic pathway?
Incactivates II - VII - IX - X - XI - XII
Collagen - BM - activated platelets
Contiguous
Petechiae

44. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
Ewing sarcoma
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
TRAP (tartrate resistant acid phosphatase

45. Why can newborns with sickle cell be asymptomatic
2 to 10
Inc HbF and dec HbS
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Plasma cell

46. What is the effected enzyme in acute intermittment porphyria?
120 days
Intravascular hemolysis
B12 def
Porphobilinogen deaminase aka uroporphyrinogen I synthase

47. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Diffuse Large b cell lymphoma
EBV
Inducers of primary antibody response
Intravascular hemolysis

48. normal RBCs added to patient's serum
Indirect coombs - agglutinate if serum anti RBC surface Ig
Hydroxyurea - bone marrow transplant
Ferrocheletase and ALA dehydrogenase
Degrades fibrin mesh and converts C3 to C3a

49. What are the variants of the RS cells
Lacunar in nodular sclerosing variant
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Nl PT - elevated PTT - intrinsic pathway defect
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

50. philadelphia chromosome - blood looks like marrow
All
Rhogam - Rh antigen immunoglobulin
CML
Lacunar in nodular sclerosing variant