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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 is the philadelphia chromosome
Sideroblastic anemia
Tissue factor converst the VII and VIIa
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
T(9;22) bcr abl

2. What symptoms are associated with Hodgkin lymphoma
HIV or immunosupression
B symptoms - fever night sweats - weight loss
No platelet clumping
Extravascular

3. discrete tumor masses arising from lymph nodes
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Lymphoma
Uroporphyrin (tea colored urine)
Incactivates II - VII - IX - X - XI - XII

4. What does antithrombin do and What activates it?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Induces differentiation of myeloblasts
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Multiple myeloma

5. What is the tx for vWD
Valine for glutamate
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
DDAVP (desmopressin) which releases stored vWF stored in endothelium
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

6. Basophilica stippling
Collagen - BM - activated platelets
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Inc RBC - dec O2 sat - inc EPO
Schistocytes and inc LDH

7. What are the four levels of alpha thal?
Beta chain underproduced - asymptomatic - heterozygote
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

8. What are the presenting symptoms of acute intermittent porphyria
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Porphyrias
ADP and Ca

9. What do you see on peripheral smear with sideroblastic anemia
Ringed sideronblasts with iron laden mitochondria
Rhogam - Rh antigen immunoglobulin
GpIIb/IIIa
20 to 40

10. What is the life spance of a platelet?
Macrohemorrhage - hemarthroses - easy bruising
40 to 50
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
8-10 days

11. Serum iron - transferrin - ferritin lab values for hemochromatosis
Inc UCB
Up - down - up
Direct coombs - agglutinate if RBCs are coated with Ig
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

12. universal recipient
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
AB - no antibodies
Extravascular hemolysis
Bone marrow - thymus - blood (80% of circulating lymphos are T)

13. What do eosinophils defend against and What do they use to do it?
Helminth infections major basic protein
Africa = Jaw lesion - US = pelvis or abdomen
40 to 50
Extravascular

14. Which factor does vWF carry/protect
CD15 and CD30 pos
Imatinib - anti bcr abl
VIII
Factor V Leiden

15. What is the most common hodgkin lymphoma
Dec synthesis of factors 1972 - protein C/S
Parvovirus
Antigen - antibody
Nodular sclerosing

16. Blood type A
G6PD
1 to 6
A antigen and B antibodies
8-10 days

17. What is the clinical picture of hemophilia A or B
Macrohemorrhage - hemarthroses - easy bruising
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
HbSS

18. Where do you see hypersegmented polys?
Spleen
VWF carries/protects factor VIII
B12/folate def
Ankryin - band 4.1 or spectrin

19. Which immunoglobulin is involved in warm agglutination?
Essential thrombocytosis
IgG - Warm is GREAT
5- FU - AZT - hydroxyurea
Africa = Jaw lesion - US = pelvis or abdomen

20. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Protoporphyrin (blood)
Leukemia
Thrombotic thrombocytopenic purpura
Protein C or S deficiency

21. X linked - dec glutathione inc RBC susceptibility to oxidative stress
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Antigen - antibody
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
G6PD

22. Target cell

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23. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Fibrinogen
Inc
Complications of sickle cell anemia
EBV

24. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Inc large vWF multimers - inc platelet aggregation and thrombosis
Hodgkin
B symptoms - fever night sweats - weight loss
VWF carries/protects factor VIII

25. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
Sickle cell
Sickle cell anemia
Fe def - thal - pb poison - sideroblastic anemia
Uroporphyrinogen decarboxylase

26. What is their role?
Inducers of primary antibody response
Histaminase and arylsulfatase
Glanzmann's throbmasthenia
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

27. What is the effected enzyme in acute intermittment porphyria?
Incactivates II - VII - IX - X - XI - XII
Multiple myeloma
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Porphobilinogen deaminase aka uroporphyrinogen I synthase

28. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Ewing sarcoma
Heinz bodies - seen in alpha thal and G6PD
Factor V Leiden
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 -

29. What is the general pathology of a macrocytic anemia?
All
G6PD
Aspirin
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

30. Sickle cell
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Sickle cell anemia
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Inc large vWF multimers - inc platelet aggregation and thrombosis

31. What converts plasminogen to plasm and What does plasmin do?
T(9;22) bcr abl
HIV or immunosupression
TPA - cleavage of fibrin mesh
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

32. anisocytosis
Inc large vWF multimers - inc platelet aggregation and thrombosis
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
VWF and fibrinogen
Varying sizes

33. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Down - down - up
Valine for glutamate
Folate def
No

34. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
PGI2 - NO inc blood flow - dec platelet aggregation
Anti Rh
Signs and sx of aplastic anemia
Leukemoid reaction

35. Which drugs can cause macrocytic anemia?
Inc HbA2 on electrophoresis
ATIII def
A antigen and B antibodies
5- FU - AZT - hydroxyurea

36. What is the pathogenesis of TTP?
Inc lymphoctes - and less RS cells
Pos
Inc large vWF multimers - inc platelet aggregation and thrombosis
Up - down - up

37. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Bernard soulier
Bleeding - malnutrition/absorption - inc demand pregs
Folate def
CML

38. Bite cell
Common a few days after oxidative stress in a pt with G6PD
Up - down - up
Ewing sarcoma
G6PD

39. What causes renal papillary necrosis in sickle cell
Uroporphyrinogen decarboxylase
Low O2 in papilla; can also get microhematuria from medullary infarcts
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Idiopathic thrombocytopenia (ITP)

40. Is G6PD intravascular or extravascular
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
B6
Howell Jolly bodies
Intravascular

41. What is the receptor for fibrinogen?
GpIIb/IIIa
G6PD
Her next Rh+ fetus
HbSS

42. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
B12 in fatty acid pathways leads to subacute combined degeneration
B12 def
Decrease EPO
They bind vWF via GpIb

43. How are the nucleus and the cytoplasm characterized for lymphocytes
Macrohemorrhage - hemarthroses - easy bruising
Rhogam - Rh antigen immunoglobulin
Round densly staining nucleus with a small amount of pale cytoplasm
Lymphoma

44. Ringed sideroblasts
Glycine and succinyl - coa
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Porphyrias
Sideroblastic anemia

45. Elderly - mature b cell tumor with filamentous - hairlike projections
Hairy cell leukemia
Def in factor IX
Dendritic cells?
IFN gama

46. drug that inhibits ADP induced expression of GpIIb/IIIa
Ticlopidine/clopidogrel
ATIII def
Protein S - cleaves and invactivates Va and VIIIa
Cromolyn sodium

47. Serum iron - transferrin - ferritin lab values for iron def anemia
Up - down - nl
Splicing sites and promotor sequences
No antigen - both antibodies
Down - up - down

48. What do labs show in ITP?
Complications of sickle cell anemia
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Acts to stabilize platelet plug
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

49. What is the tx for aplastic anemia
Bone marrow - thymus - blood (80% of circulating lymphos are T)
M3 AML (acute promyelocytic leukemia)
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Inc vasodiltion - inc perm - inc pain

50. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
120 days
Pyruvate kinase def - extravascular
Her next Rh+ fetus
All