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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. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Inc UCB
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

2. Elderly - mature b cell tumor with filamentous - hairlike projections
PGI2 - NO inc blood flow - dec platelet aggregation
5- FU - AZT - hydroxyurea
Hairy cell leukemia
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

3. What virus can cause an aplastic crisis in pts with HS?
Epoxide reductase - warfarin inhibits
Parvovirus
Protoporphyrin
Inc large vWF multimers - inc platelet aggregation and thrombosis

4. What is the tx for aplastic anemia
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
MAHA
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Alpha thal - asian and african american

5. What is the therapy for the M3 variant?
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Inc serum iron - normal TIBC - inc ferratin
All trans retinoic acid
Leukemia

6. What does STOP Making New Thrombi stand for
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Hereditary spherocytosis - autoimmune hemolysis
Ankryin - band 4.1 or spectrin
Abciximab

7. Plasma cell neoplasm
Inc lymphoctes - and less RS cells
Multiple myeloma
Indirect coombs - agglutinate if serum anti RBC surface Ig
Protoporphyrin

8. X linked - dec glutathione inc RBC susceptibility to oxidative stress
G6PD
IgG - Warm is GREAT
Uroporphyrin (tea colored urine)
Beta 4

9. Which factor does vWF carry/protect
VIII
Fibrinogen bind GpIIb/IIIa and links platelts
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Beta 4

10. Does CML have a JAK2 mut
IFN gama
Tissue
No
Macrohemorrhage - hemarthroses - easy bruising

11. anti - Ig antibody added to patients RBCs;
Hydroxyurea - bone marrow transplant
HIV or immunosupression
Helps platelts adhere to endothelium
Direct coombs - agglutinate if RBCs are coated with Ig

12. Is G6PD intravascular or extravascular
Intravascular
Acts to stabilize platelet plug
Helps platelts adhere to endothelium
CML to AML or All

13. What are the neuro sx of B12 def?
Leukemia
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
No platelet clumping
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

14. What reveresible things can a sideroblastic anemia
Uroporphyrinogen decarboxylase
Lead - and EtOH
Inc HbF and dec HbS
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

15. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Ankryin - band 4.1 or spectrin
No platelet clumping
Histamine - heparin - and eosinohil chemotactic factors
Leukemia

16. What are the etiologies of folate def
Sideroblastic anemia
Intrinsic - all factors except - VII - XIII
Helminth infections major basic protein
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

17. adults - auer rods - inc circulating myeblasts 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 -
Diffuse Large b cell lymphoma
Idiopathic thrombocytopenia (ITP)
AML

18. What virus is associated with 50% of Hodgkin lymphoma
Anemia of chronic disease - Aplastic anemia - kidney disease
Easy gas exchange
EBV
Acts to stabilize platelet plug

19. mother's antibodies attack fetal RBCs
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Nl - up - nl
Von Willebrand's disease
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

20. What does the large SA:volume ratio in RBCs help facilitate?
Anemia of chronic disease - Aplastic anemia - kidney disease
Round densly staining nucleus with a small amount of pale cytoplasm
Easy gas exchange
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

21. What is appropriate absolute polycythemia
Hereditary spherocytosis - G6PD - sickle cell
Mycosis fundgoides/Sezary syndrome
Inc RBC - dec O2 sat - inc EPO
Hereditary spherocytosis - autoimmune hemolysis

22. What does NAACP stand for - in regards to the causes of eosinophiia?
Plummer - vinson syndrome
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
2 to 10
B symptoms - fever night sweats - weight loss

23. What is factor V leidin?

24. What is the activation stage of platelet plug formation?
Examples of oxidative stress
Protoporphyrin (blood)
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

25. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
Hereditary elloptocytosis
Sideroblastic anemia
Down - up - down

26. Who are the professional APCs?
Dendritic cells?
Hereditary spherocytosis - autoimmune hemolysis
Rhogam - Rh antigen immunoglobulin
Valine for glutamate

27. bcl -2 t(14;18) adults
Follicular lymphoma - indolent course
Fibrinogen
VWF and fibrinogen
Nodular sclerosing

28. From what cells are platelets derived from
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Megakaryocytes
No

29. Target cell

30. What are the labs and tx for HS?
Positive osmootic fragility test and splenectomy
Inc RBC - dec O2 sat - inc EPO
Extrinsic - I - II - V - VII and X
Megakaryocytes

31. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Heinz bodies - seen in alpha thal and G6PD
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Produce antibody - lots of RER and golgi
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

32. What percentage if WBCs are polys?
40 to 75
Blod - tissue - MACS
Petechiae
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

33. Blood type B
Idiopathic thrombocytopenia (ITP)
Liver disease - abetalipoproteinemia - acntho = spiny
Polycythemia vera
B antigena and A antibodes

34. What is the are the presenting symptoms of lead poisoning in kids and adults
Cromolyn sodium
Ticlopidine/clopidogrel
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

35. What the alpha granules contain in platelets?
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
PGI2 - NO inc blood flow - dec platelet aggregation
HbSS
VWF and fibrinogen

36. What substance prevents mast cells degranulation?
Hairy cell leukemia
Blasts > 5%
Cromolyn sodium
Round densly staining nucleus with a small amount of pale cytoplasm

37. What are the four levels of alpha thal?
T(12;21)
Low in CML
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Howell Jolly bodies

38. What is the accumulated substance in lead poisoning
Protoporphyrin (blood)
TPA - cleavage of fibrin mesh
Cromolyn sodium
B12/folate def

39. Serum iron - transferrin - ferritin lab values for hemochromatosis
CML
Howell - Jolly bodies - hypo/asplenia
Acts to stabilize platelet plug
Up - down - up

40. What is monoclonal expansion without symptoms associated with multiple myeloma?
PGI2 - NO inc blood flow - dec platelet aggregation
MGUS - monoclonal gammopathy of undetermined significance
Valine for glutamate
Protein C or S deficiency

41. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Indirect coombs - agglutinate if serum anti RBC surface Ig
Epoxide reductase - warfarin inhibits
Hodgkin
Lung disease - congenital heart diseaes - and high altitude

42. What are dendritic cells called in the skin?
Hb Barts - gamma4 in defect in all 4 alpha genes
Pos
Petechiae
Langerhans cells

43. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
AB - no antibodies
Kids= exposure to lead paint - adults = battery - ammunition factory
Down - down - up

44. What is the therapy for CML (philly chrom)
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Fibrinogen bind GpIIb/IIIa and links platelts
Imatinib - anti bcr abl
AML

45. When is the peak incidence for nonHod lymphoma
Mantle cell lymphoma
Inc vasodiltion - inc perm - inc pain
20 to 40
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

46. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
20 to 40
Idiopathic thrombocytopenia (ITP)
Folate def
Antigen - antibody

47. S-100 and CD1a with birbeck granules
Aspirin
Langerhans cell histiocytosis
Schistocytes - helmet cells
M3 AML (acute promyelocytic leukemia)

48. What do you see in peripheral smear in a pt with G6PD?
Imatinib - anti bcr abl
T(9;22) bcr abl
Bite cells and Heinz bodies
Langerhans cells

49. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Inc RBC - dec O2 sat - inc EPO
Nl - up - nl
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Helminth infections major basic protein

50. Mutation in 3' untranslated region associated with venous clots
Splicing sites and promotor sequences
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Prothrombin gene mutation
Hb Barts - gamma4 in defect in all 4 alpha genes