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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. Who has more severe disease - HbSS or HbSC
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
HbSS
Imatinib - anti bcr abl

2. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Eleveated PT - PTT
MHC II and Fc receptors
Beta thal

3. What does 'Neutrophils Like Making Everything Better' stand for?
Blistering cutaneous photosens - most common porphyria
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Antigen - antibody
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

4. What are the neuro sx of B12 def?
Orotic aciduria
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
VWF and fibrinogen

5. What indicated a good prognosis in Hodgkin lymphoma?
Inc lymphoctes - and less RS cells
Protein S - cleaves and invactivates Va and VIIIa
Bimodal - men>women - except for nodular sclerosing type
Myelofibrosis (marow is crying being its fibrosed'

6. What is the affected enzyme in acute intermittment porphyria
Rhogam - Rh antigen immunoglobulin
Porphobilinogen - delta ALA - uroporphyrin
Def in factor IX
Yes - via MHC II

7. Bite cell
G6PD
Epoxide reductase - warfarin inhibits
Abciximab
Liver disease - abetalipoproteinemia - acntho = spiny

8. Fibrotic obliteration of bone marow with teardrop cells

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9. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
TPA - cleavage of fibrin mesh
Ewing sarcoma
Signs and sx of aplastic anemia
Common a few days after oxidative stress in a pt with G6PD

10. Serum iron - transferrin - ferritin lab values for iron def anemia
Signs and sx of aplastic anemia
No lytic bone lesions in WM
Howell Jolly bodies
Down - up - down

11. What are some classic examples of extravascular hemolysis
Inc
Malaria - Babesia
Hereditary spherocytosis - G6PD - sickle cell
Def in factor IX

12. universal donor
Factor V Leiden
No antigen - both antibodies
Leukemia
Sickle cell

13. What is the tx for aplastic anemia
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
ADP and Ca
Her next Rh+ fetus
Exposed collagen upon endothelial damage

14. What is relative polycythemia
40 to 75
Leukemoid reaction
Gp1b
Dec plasma volume

15. what proteins can be defective in HS?
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Ankryin - band 4.1 or spectrin

16. What causes hydrops fetalis
G6PD
MHC II and Fc receptors
DIC - TTP/HUS - traumatic hemolysis
Hb Barts - gamma4 in defect in all 4 alpha genes

17. What causes renal papillary necrosis in sickle cell
Low O2 in papilla; can also get microhematuria from medullary infarcts
Direct coombs - agglutinate if RBCs are coated with Ig
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Adult T cell lymphoma - presents with cutaneous lesions

18. Upregulated growth of leukocytes in bone marro
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Hodgkin
Dec synthesis of factors 1972 - protein C/S
Leukemia

19. What does hairy cell leukemia stain with
HbSS
Varying shapes
TRAP (tartrate resistant acid phosphatase
ATIII def

20. adults - auer rods - inc circulating myeblasts on peripheral smear
DIC
Mantle cell lymphoma
AML
20 to 40

21. What is the philadelphia chromosome
Fc
Blistering cutaneous photosens - most common porphyria
T(9;22) bcr abl
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

22. What is the life spance of a platelet?
Lacunar in nodular sclerosing variant
Dec synthesis of factors 1972 - protein C/S
Schistocytes and inc LDH
8-10 days

23. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Fibrinogen
All
Anemia of chronic disease - Aplastic anemia - kidney disease
G6PD

24. What does bradykinin do?
No antigen - both antibodies
Inc vasodiltion - inc perm - inc pain
HbSS
TPA - cleavage of fibrin mesh

25. Elderly - mature b cell tumor with filamentous - hairlike projections
Tissue
Hairy cell leukemia
Ankryin - band 4.1 or spectrin
Rhogam - Rh antigen immunoglobulin

26. Elliptocyte
Tissue
Incactivates II - VII - IX - X - XI - XII
Hereditary elloptocytosis
20 to 40

27. Why does B12 def cause neuro sx?
B12 in fatty acid pathways leads to subacute combined degeneration
Inappropriate absolute with inc RBCs and EPO
Aspirin
Off center nuclues - clock face chromatin

28. Target cell

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29. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Lacunar in nodular sclerosing variant
Complications of sickle cell anemia
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

30. What condition can result from treating AML M3 from the release of the Auer rods
Von Willebrand's disease
DIC
HIV or immunosupression
G6PD

31. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Direct coombs - agglutinate if RBCs are coated with Ig
Inappropriate absolute with inc RBCs and EPO
ADP and Ca
CLL (SLL without the peripheral lymphocytosis

32. What do plasma cells do
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Produce antibody - lots of RER and golgi
Down - down - up
Extravascular hemolysis

33. t(11;14)
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
They bind vWF via GpIb
Mantle cell lymphoma
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

34. What are the presenting symptoms of acute intermittent porphyria
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Both alpha and beta thal
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Thrombotic thrombocytopenic purpura

35. From what cells are platelets derived from
PGI2 - NO inc blood flow - dec platelet aggregation
Produce antibody - lots of RER and golgi
Megakaryocytes
EBV

36. What is the therapy for the M3 variant?
All trans retinoic acid
Extravascular hemolysis
DIC
Inc serum iron - normal TIBC - inc ferratin

37. What is the pathogenesis of aplastic anemia with kidney disease
Blasts > 5%
Decrease EPO
Blood
...

38. What are the likely exposures of kids and adults for lead poisoning
Positive osmootic fragility test and splenectomy
PGI2 - NO inc blood flow - dec platelet aggregation
Kids= exposure to lead paint - adults = battery - ammunition factory
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

39. tennis rackets on EM
40 to 75
Birbeck granules
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
EBV

40. What does Vit K deficiency cause?
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Dec synthesis of factors 1972 - protein C/S
AB - no antibodies
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

41. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Protoporphyrin (blood)
Beta thal
Ankryin - band 4.1 or spectrin
Folate def

42. What do the platelets bind? What is the step called
Direct coombs - agglutinate if RBCs are coated with Ig
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 -
They bind vWF via GpIb

43. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
Histaminase and arylsulfatase
Heinz bodies - seen in alpha thal and G6PD
All trans retinoic acid
ATIII def

44. What chromosomal translocation is associated with a better prognosis in All
Inc RBC - dec O2 sat - inc EPO
T(12;21)
Ringed sideronblasts with iron laden mitochondria
40 to 50

45. iron deficiency anemia - esophageal web - atrophic glossitis
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Plummer - vinson syndrome
No platelet clumping
Hereditary spherocytosis

46. t(8;14) c - myc gene
Signs and sx of aplastic anemia
8-10 days
Burkitt lymphoma
Inappropriate absolute with inc RBCs and EPO

47. What is the treatment for sideroblastic anemia
B6 therapy (pyrodixine)
AB - no antibodies
Incactivates II - VII - IX - X - XI - XII
Leukemia

48. Causes of iron def?
Africa = Jaw lesion - US = pelvis or abdomen
Ticlopidine/clopidogrel
Bleeding - malnutrition/absorption - inc demand pregs
Porphobilinogen - delta ALA - uroporphyrin

49. Which immunoglobulin is involved in warm agglutination?
TRAP (tartrate resistant acid phosphatase
IgG - Warm is GREAT
Glanzmann's throbmasthenia
Intravascular

50. Mutation in 3' untranslated region associated with venous clots
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
VWF and fibrinogen
Liver disease - abetalipoproteinemia - acntho = spiny
Prothrombin gene mutation

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