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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. Where does All spread
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
CNS and testis
Protein C or S deficiency
5- FU - AZT - hydroxyurea

2. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
No
Down - up - down
Leukemia
Paraprotein spike - monoclonal protein

3. Who has more severe disease - HbSS or HbSC
MGUS - monoclonal gammopathy of undetermined significance
HbSS
Aspirin
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

4. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
Porphobilinogen - delta ALA - uroporphyrin
Burkitt lymphoma
All
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

5. Upregulated growth of leukocytes in bone marro
Low O2 in papilla; can also get microhematuria from medullary infarcts
Leukemia
Hereditary spherocytosis
EBV

6. What is monoclonal expansion without symptoms associated with multiple myeloma?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
MGUS - monoclonal gammopathy of undetermined significance
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Hereditary spherocytosis - autoimmune hemolysis

7. Which cell is neoplastic in multiple myeloma
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
Plasma cell
Prothrombin gene mutation

8. What is their role?
Inc RBC - dec O2 sat - inc EPO
Lacunar in nodular sclerosing variant
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Inducers of primary antibody response

9. Fibrotic obliteration of bone marow with teardrop cells

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10. What is the affected enzyme in lead poisoning
No platelet clumping
Ferrocheletase and ALA dehydrogenase
Valine for glutamate
Fibrinogen

11. Deficiency in vWF

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12. Macro - ovalocyte
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Leukemia
Her next Rh+ fetus
Acts to stabilize platelet plug

13. What does the blood smear show in glanzmann's?
Extrinsic - I - II - V - VII and X
Inc HbA2 on electrophoresis
Antigen - antibody
No platelet clumping

14. What substance prevents mast cells degranulation?
Bimodal - men>women - except for nodular sclerosing type
Cromolyn sodium
Leukemia
Low in CML

15. What are the presenting symptoms of acute intermittent porphyria
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
M3 AML (acute promyelocytic leukemia)
Essential thrombocytosis
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

16. What is the receptor for vWF
5- FU - AZT - hydroxyurea
EBV
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Gp1b

17. What is the Ddx for nonhemolytic normocytic anemia
Eleveated PT - PTT
Intravascular hemolysis
B symptoms - fever night sweats - weight loss
Anemia of chronic disease - Aplastic anemia - kidney disease

18. What is the age group most commonly affected by multiple myeloma
Hodgkin
40 to 50
Blod - tissue - MACS
DDAVP (desmopressin) which releases stored vWF stored in endothelium

19. Conditions of defective heme synthesis leading to accumulation of heme precurors
Sideroblastic anemia
Porphyrias
Ankryin - band 4.1 or spectrin
Protein S - cleaves and invactivates Va and VIIIa

20. Mutation in 3' untranslated region associated with venous clots
Schistocytes and inc LDH
Acts to stabilize platelet plug
T(12;21)
Prothrombin gene mutation

21. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Sideroblastic anemia
Inc RBC - dec O2 sat - inc EPO
Intravascular hemolysis
Round densly staining nucleus with a small amount of pale cytoplasm

22. What does increasing heme do to ALA synthase activity
...
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 -
Dec
HIV or immunosupression

23. What are the pro aggregation factors?
TXA2 - dec blood flow - inc platelet aggregation
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Both alpha and beta thal
Extravascular

24. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Splicing sites and promotor sequences
M3 AML (acute promyelocytic leukemia)
Down - down - up
Dec

25. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Multiple myeloma
Tissue factor converst the VII and VIIa
Anti Rh
Up - down - nl

26. t(8;14) c - myc gene
Antigen - antibody
Beta thal
Burkitt lymphoma
Ticlopidine/clopidogrel

27. Deficiency in GpIb
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Round densly staining nucleus with a small amount of pale cytoplasm
Blod - tissue - MACS
Bernard soulier

28. Where are 1/3 of platelets stored
EBV
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Polycythemia vera
Spleen

29. Where do B cells arise from - mature - and migrate to...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
20 to 40
B antigena and A antibodes
Spleen

30. CD5+ - poor prognosis - t(11;14)
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Africa = Jaw lesion - US = pelvis or abdomen
Nl PT - elevated PTT - intrinsic pathway defect
Mantle cell - older males

31. What substance accumulates in porphyria cutanea
SLE - CLL - alpha methyldopa
Extravascular hemolysis
Polycythemia vera
Uroporphyrin (tea colored urine)

32. How do platelet disorders present?
Decrease EPO
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Beta chain underproduced - asymptomatic - heterozygote

33. What are the age ranges for the various leukemias
Cromolyn sodium
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
All trans retinoic acid
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

34. What is the therapy for the M3 variant?
Rhogam - Rh antigen immunoglobulin
8-10 days
All trans retinoic acid
Inc HbF and dec HbS

35. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Dendritic cells?
DIC
Idiopathic thrombocytopenia (ITP)

36. Blood type A
Ankryin - band 4.1 or spectrin
A antigen and B antibodies
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Pos

37. Spherocyte
Fibrinogen bind GpIIb/IIIa and links platelts
Hereditary spherocytosis - autoimmune hemolysis
40 to 50
B12/folate def

38. What are the main associations with multiple myeloma?
Myelofibrosis (marow is crying being its fibrosed'
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Hereditary spherocytosis - G6PD - sickle cell
Indirect coombs - agglutinate if serum anti RBC surface Ig

39. What is the tx for aplastic anemia
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Helminth infections major basic protein
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

40. What is the most common hodgkin lymphoma
PGI2 - NO inc blood flow - dec platelet aggregation
Diffuse Large b cell lymphoma
...
Nodular sclerosing

41. What is hemophilia B?
Def in factor IX
Dec synthesis of factors 1972 - protein C/S
Orotic aciduria
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

42. What is a blast crisis
Thrombotic thrombocytopenic purpura
CML to AML or All
Pos
Protoporphyrin (blood)

43. Elliptocyte
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Hereditary elloptocytosis
PGI2 - NO inc blood flow - dec platelet aggregation
Direct coombs - agglutinate if RBCs are coated with Ig

44. Basophilic nuclear remnants fonud in RBCs
M3 AML (acute promyelocytic leukemia)
Eleveated PT - PTT
Reed - sternberg cells
Howell - Jolly bodies - hypo/asplenia

45. When is the peak incidence for nonHod lymphoma
20 to 40
Produce antibody - lots of RER and golgi
Dec
Inducers of primary antibody response

46. What do labs show in DIC?
MAHA
Inc lymphoctes - and less RS cells
Incactivates II - VII - IX - X - XI - XII
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

47. What cell is primarily involved in non Hod lymph
B cells
Inducers of primary antibody response
CML
T(9;22) bcr abl

48. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Histaminase and arylsulfatase
Lymphoma
Varying shapes

49. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Pyruvate kinase def - extravascular
B12 def
Fe def - thal - pb poison - sideroblastic anemia
Her next Rh+ fetus

50. What does antithrombin do and What activates it?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
TXA2 - dec blood flow - inc platelet aggregation
Mycosis fundgoides/Sezary syndrome
Nl - up - nl

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Hemeonc

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