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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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1. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Factor V Leiden
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Idiopathic thrombocytopenia (ITP)

2. Elliptocyte
Mantle cell lymphoma
Hereditary elloptocytosis
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Birbeck granules

3. What is the main source of energy in RBCs
Hb Barts - gamma4 in defect in all 4 alpha genes
90% anearobically from glucose to lactate - 10% from HMP shunt
Diffuse Large b cell lymphoma
SLE - CLL - alpha methyldopa

4. adults - auer rods - inc circulating myeblasts on peripheral smear
Anemia of chronic disease - Aplastic anemia - kidney disease
Ringed sideronblasts with iron laden mitochondria
AML
Lysine for glutamate at position 6

5. What does hairy cell leukemia stain with
Blistering cutaneous photosens - most common porphyria
TRAP (tartrate resistant acid phosphatase
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
B6

6. What percentage if WBCs are polys?
40 to 75
Bernard soulier
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Porphyrias

7. hypocellular bone marrown tih fatty infiltration
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Essential thrombocytosis
Beta chain underproduced - asymptomatic - heterozygote
Aplastic anemia - pancytopenia

8. What are the anti aggregation factors?
Pos
Ewing sarcoma
Hereditary spherocytosis
PGI2 - NO inc blood flow - dec platelet aggregation

9. What is the therapy for the M3 variant?
All trans retinoic acid
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)
Leukemoid reaction

10. What are some causes of cold agglutinin anemia
Epoxide reductase - warfarin inhibits
AB - no antibodies
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
All

11. What is the tx for aplastic anemia
Essential thrombocytosis
Eleveated PT - PTT
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Induces differentiation of myeloblasts

12. What is makes a leukemia acute
Blasts > 5%
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Diffuse Large b cell lymphoma
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

13. inc LDH - jaundice
Extravascular hemolysis
Intrinsic - all factors except - VII - XIII
Idiopathic thrombocytopenia (ITP)
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

14. What is contained within the azurophilic granules of PMNs
Protoporphyrin (blood)
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Blod - tissue - MACS
Blistering cutaneous photosens - most common porphyria

15. Spherocyte
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Bite cells and Heinz bodies
Hereditary spherocytosis - autoimmune hemolysis
Dec

16. What does 'Neutrophils Like Making Everything Better' stand for?
Burkitt lymphoma
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Pyruvate kinase def - extravascular
Intravascular

17. What do the dense granules contain in platelets
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Glanzmann's throbmasthenia
ADP and Ca
B6

18. what proteins can be defective in HS?
All
Ankryin - band 4.1 or spectrin
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Complications of sickle cell anemia

19. How do platelet disorders present?
Ferrocheletase and ALA dehydrogenase
Imatinib - anti bcr abl
IFN gama
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

20. What is the tx for vWD
Eleveated PT - PTT
...
DDAVP (desmopressin) which releases stored vWF stored in endothelium
B6 therapy (pyrodixine)

21. RBCs damagaed from passing through obstructed - narrowed vessel lumina
AML
MAHA
DIC - TTP/HUS - traumatic hemolysis
Pyruvate kinase def - extravascular

22. What is the accumulated substance in lead poisoning
Blistering cutaneous photosens - most common porphyria
Protoporphyrin (blood)
Lysosomes
Sideroblastic anemia

23. Mutation in 3' untranslated region associated with venous clots
Easy gas exchange
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Prothrombin gene mutation
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

24. What chromosomal translocation is associated with a better prognosis in All
T(12;21)
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
DIC
Inc

25. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Anti Rh
CLL (SLL without the peripheral lymphocytosis
Paraprotein spike - monoclonal protein
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

26. What substance is Fe added to to yield heme
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Alpha thal - asian and african american
Hb Barts - gamma4 in defect in all 4 alpha genes
Protoporphyrin

27. How does the therapy for M3 vairant work?
CML
Glucose and heme - which inhibit ALA synthase
Complications of sickle cell anemia
Induces differentiation of myeloblasts

28. Fava beans - sufla drugs - infectinos
Examples of oxidative stress
Von Willebrand's disease
Tissue factor converst the VII and VIIa
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

29. What is hemophiliia A
Deficiency in factor VIII
2 to 10
Inc large vWF multimers - inc platelet aggregation and thrombosis
Hb Barts - gamma4 in defect in all 4 alpha genes

30. What is the treatment for sideroblastic anemia
Beta chain underproduced - asymptomatic - heterozygote
M3 AML (acute promyelocytic leukemia)
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
B6 therapy (pyrodixine)

31. What finding you do you see in patients after splenectomy
Howell Jolly bodies
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
No antigen - both antibodies
VIII

32. What is the life span of a normal RBC
Alpha thal - asian and african american
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
120 days

33. What is the general pathology of a macrocytic anemia?
Extrinsic - I - II - V - VII and X
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
G6PD
Dec plasma volume

34. What is the are the presenting symptoms of lead poisoning in kids and adults
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Degrades fibrin mesh and converts C3 to C3a

35. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Lysosomes
Nodular sclerosing
Degrades fibrin mesh and converts C3 to C3a
Protein C or S deficiency

36. 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 -
Lysine for glutamate at position 6
Protein C or S deficiency
Degrades fibrin mesh and converts C3 to C3a

37. What vitamin is a cofactor for the first step of heme synthesis
Yes - via MHC II
Blod - tissue - MACS
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
B6

38. Which drugs can cause macrocytic anemia?
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Round densly staining nucleus with a small amount of pale cytoplasm
5- FU - AZT - hydroxyurea
Factor V Leiden

39. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Nl - up - nl
1 to 6
40 to 50
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

40. What is the purpose of the fibrin mesh
Lysine for glutamate at position 6
Alpha thal - asian and african american
B6 therapy (pyrodixine)
Acts to stabilize platelet plug

41. What do auer rods stain with
5- FU - AZT - hydroxyurea
All
Peroxidase
MGUS - monoclonal gammopathy of undetermined significance

42. Is HS extravascular or intravascular?
Yes - via MHC II
Aspirin
Extravascular
PGI2 - NO inc blood flow - dec platelet aggregation

43. Where does All spread
Low in CML
CNS and testis
T(12;21)
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

44. What causes the physiologic chloride shift and What does the chloride shift do?
Alpha thal - asian and african american
Schistocytes - helmet cells
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Ticlopidine/clopidogrel

45. What is the activation stage of platelet plug formation?
Inappropriate absolute with inc RBCs and EPO
Anti Rh
Plummer - vinson syndrome
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

46. What happens in betal thal minor?
Beta chain underproduced - asymptomatic - heterozygote
CML
Langerhans cell histiocytosis
Sideroblastic anemia

47. Why can newborns with sickle cell be asymptomatic
SLE - CLL - alpha methyldopa
Inc HbF and dec HbS
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Porphobilinogen - delta ALA - uroporphyrin

48. iron deficiency anemia - esophageal web - atrophic glossitis
MGUS - monoclonal gammopathy of undetermined significance
Fibrinogen bind GpIIb/IIIa and links platelts
Alpha thal - asian and african american
Plummer - vinson syndrome

49. What is the mutation in HbC
Glanzmann's throbmasthenia
Lysine for glutamate at position 6
Lead - and EtOH
Africa = Jaw lesion - US = pelvis or abdomen

50. deficiency in ADAMTS13 leading to dec degradation of vWF
Bernard soulier
Thrombotic thrombocytopenic purpura
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Parvovirus