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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. Ddx for microcytic anemia
Lacunar in nodular sclerosing variant
Blood
Fe def - thal - pb poison - sideroblastic anemia
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

2. What is their role?
Howell Jolly bodies
Inducers of primary antibody response
Lacunar in nodular sclerosing variant
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

3. RBCs damagaed from passing through obstructed - narrowed vessel lumina
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
MAHA
Tissue
Easy gas exchange

4. What is the treatment for acute intermittent porphyria
Fe def - thal - pb poison - sideroblastic anemia
Glucose and heme - which inhibit ALA synthase
Signs and sx of aplastic anemia
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

5. Mutation in 3' untranslated region associated with venous clots
All trans retinoic acid
Multiple myeloma
ATIII def
Prothrombin gene mutation

6. What are some causes of cold agglutinin anemia
Inc large vWF multimers - inc platelet aggregation and thrombosis
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Blood
G6PD

7. t(11;14)
Mantle cell lymphoma
Parvovirus
Low in CML
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

8. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
Complications of sickle cell anemia
Aplastic anemia - pancytopenia
Inc HbA2 on electrophoresis

9. What causes renal papillary necrosis in sickle cell
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Beta thal
Low O2 in papilla; can also get microhematuria from medullary infarcts
Examples of oxidative stress

10. What do platelets interact with to form a hemostatic plug
All trans retinoic acid
MAHA
Fibrinogen
Varying shapes

11. What cell is primarily involved in non Hod lymph
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
B cells
Leukemia
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

12. What is HbH
8-10 days
Beta 4
Decrease EPO
VIII

13. Serum iron - transferrin - ferritin lab values for hemochromatosis
Up - down - up
Degrades fibrin mesh and converts C3 to C3a
Common a few days after oxidative stress in a pt with G6PD
Def in factor IX

14. inc LDH - jaundice
DIC - TTP/HUS - traumatic hemolysis
Plasma cell
ADP and Ca
Extravascular hemolysis

15. adults - auer rods - inc circulating myeblasts on peripheral smear
AML
No antigen - both antibodies
Lysosomes
Up - down - up

16. Where are 1/3 of platelets stored
Helminth infections major basic protein
Spleen
GpIIb/IIIa
TPA - cleavage of fibrin mesh

17. What is the presenting scenario for TTP?
Protein C or S deficiency
2 to 10
Bone marrow infiltration - myelofibrosis
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

18. Drug that inhbits the GpIIb/IIIa directly
DDAVP (desmopressin) which releases stored vWF stored in endothelium
B antigena and A antibodes
Abciximab
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

19. Does CML have a JAK2 mut
Fibrinogen bind GpIIb/IIIa and links platelts
Round densly staining nucleus with a small amount of pale cytoplasm
No
Inc vasodiltion - inc perm - inc pain

20. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Polycythemia vera
Inc large vWF multimers - inc platelet aggregation and thrombosis
Protoporphyrin (blood)
Signs and sx of aplastic anemia

21. What is appropriate absolyte polycythemia associated with
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Lung disease - congenital heart diseaes - and high altitude
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
B12/folate def

22. hemolytic in a newborn - dec ATP and rigid RBCs
Leukemia
Macrohemorrhage - hemarthroses - easy bruising
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Pyruvate kinase def - extravascular

23. What do eosinophils defend against and What do they use to do it?
Cromolyn sodium
Helminth infections major basic protein
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

24. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Round densly staining nucleus with a small amount of pale cytoplasm
Cromolyn sodium
Sideroblastic anemia
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

25. What do labs show in DIC?
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Def in factor IX
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Alpha thal - asian and african american

26. What does LEAD stand for in lead poisoning?
Blistering cutaneous photosens - most common porphyria
Ringed sideronblasts with iron laden mitochondria
Contiguous
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 -

27. what proteins can be defective in HS?
Intrinsic - all factors except - VII - XIII
Nl - up - nl
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Ankryin - band 4.1 or spectrin

28. What are the etiologies of B12 def
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Gp1b
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Protoporphyrin (blood)

29. What is the philadelphia chromosome
T(9;22) bcr abl
Factor V Leiden
Mantle cell lymphoma
DIC

30. What state is commonly associated with nonHod lymphoma
Low in CML
HIV or immunosupression
MHC II and Fc receptors
PGI2 - NO inc blood flow - dec platelet aggregation

31. What is the treatment for lead poising?
Lung disease - congenital heart diseaes - and high altitude
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
B antigena and A antibodes
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

32. What do you see in peripheral smear in a pt with G6PD?
Heinz bodies - seen in alpha thal and G6PD
Intravascular
Bite cells and Heinz bodies
Protein C or S deficiency

33. What does hairy cell leukemia stain with
Valine for glutamate
Ankryin - band 4.1 or spectrin
Megakaryocytes
TRAP (tartrate resistant acid phosphatase

34. Why does B12 def cause neuro sx?
GpIIb/IIIa
B12 in fatty acid pathways leads to subacute combined degeneration
20 to 40
Incactivates II - VII - IX - X - XI - XII

35. What is the Ddx for nonhemolytic normocytic anemia
T(9;22) bcr abl
CML to AML or All
Anemia of chronic disease - Aplastic anemia - kidney disease
Hereditary spherocytosis

36. What do you see in vit K def
Incactivates II - VII - IX - X - XI - XII
Rhogam - Rh antigen immunoglobulin
Eleveated PT - PTT
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

37. Causes of iron def?
EBV
Low O2 in papilla; can also get microhematuria from medullary infarcts
Bleeding - malnutrition/absorption - inc demand pregs
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

38. What does increasing heme do to ALA synthase activity
8-10 days
Anti Rh
Dec
Direct coombs - agglutinate if RBCs are coated with Ig

39. Where do you see hypersegmented polys?
Fc
Thrombotic thrombocytopenic purpura
B12/folate def
Hairy cell leukemia

40. What is the enzyme defect in Porphyria cutanea tarda
Nl - up - nl
Factor V Leiden
Uroporphyrinogen decarboxylase
Mantle cell - older males

41. Which factor does vWF carry/protect
Bone marrow infiltration - myelofibrosis
VIII
Beta thal
Both alpha and beta thal

42. bcl -2 t(14;18) adults
Follicular lymphoma - indolent course
Leukemia
Plummer - vinson syndrome
Lacunar in nodular sclerosing variant

43. What percentage if WBCs are polys?
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Inappropriate absolute with inc RBCs and EPO
40 to 75

44. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
All
Her next Rh+ fetus
Helps platelts adhere to endothelium
Alpha thal - asian and african american

45. What are the presenting symptoms of porphyria cutanea tarda
Aspirin
T(12;21)
Histamine - heparin - and eosinohil chemotactic factors
Blistering cutaneous photosens - most common porphyria

46. What is the pathogenesis of TTP?
Porphyrias
Incactivates II - VII - IX - X - XI - XII
Inc large vWF multimers - inc platelet aggregation and thrombosis
Fc

47. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
ADP and Ca
Paroxysmal nocturnal hemoglobinuria
Sideroblastic anemia
Parvovirus

48. Defect in platelet to platelet adhesion - receptor - disorder - platelet count and bleeding time

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49. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Bone marrow - thymus - blood (80% of circulating lymphos are T)
40 to 75
Protoporphyrin (blood)
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

50. Fibrotic obliteration of bone marow with teardrop cells

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