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Hemeonc

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1. What is the therapy for the M3 variant?
All trans retinoic acid
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
CD15 and CD30 pos
B12 in fatty acid pathways leads to subacute combined degeneration

2. Plasma cell neoplasm
VWF carries/protects factor VIII
Porphyrias
Multiple myeloma
DIC

3. What percentage if WBCs are polys?
Helps platelts adhere to endothelium
VWF carries/protects factor VIII
40 to 75
Anti Rh

4. Deficiency in vWF

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5. What converts plasminogen to plasm and What does plasmin do?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
TPA - cleavage of fibrin mesh
Blasts > 5%

6. What indicated a good prognosis in Hodgkin lymphoma?
Inc lymphoctes - and less RS cells
No
Leukemoid reaction
Ticlopidine/clopidogrel

7. Ddx for microcytic anemia
Fe def - thal - pb poison - sideroblastic anemia
Aplastic anemia - pancytopenia
T(9;22) bcr abl
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

8. What is the characteristic spread of Hodgkin Lymphoma
Extravascular
Contiguous
Hereditary elloptocytosis
Decrease EPO

9. What is the age group most commonly affected by multiple myeloma
Alpha thal - asian and african american
Anemia of chronic disease - Aplastic anemia - kidney disease
40 to 50
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

10. What does Vit K deficiency cause?
CNS and testis
Leukemoid reaction
Mantle cell lymphoma
Dec synthesis of factors 1972 - protein C/S

11. inc LDH - jaundice
Inc HbA2 on electrophoresis
Extravascular hemolysis
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Leukemoid reaction

12. t(11:22)
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Essential thrombocytosis
Uroporphyrinogen decarboxylase
Ewing sarcoma

13. What are the presenting symptoms of porphyria cutanea tarda
Histamine - heparin - and eosinohil chemotactic factors
Blistering cutaneous photosens - most common porphyria
Leukemia
Africa = Jaw lesion - US = pelvis or abdomen

14. What is the general pathology of a macrocytic anemia?
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Complications of sickle cell anemia
Induces differentiation of myeloblasts
No platelet clumping

15. Fava beans - sufla drugs - infectinos
Dendritic cells?
AB - no antibodies
Inc vasodiltion - inc perm - inc pain
Examples of oxidative stress

16. What are the main associations 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)
Peroxidase
Abciximab
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

17. What begins the extrinsic pathway?
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Intravascular hemolysis
TRAP (tartrate resistant acid phosphatase
Tissue factor converst the VII and VIIa

18. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
DIC
Aspirin
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Sickle cell anemia

19. What is HbH
Beta 4
Intrinsic - all factors except - VII - XIII
Mycosis fundgoides/Sezary syndrome
Tissue

20. What happens in betal thal minor?
CML
B12 in fatty acid pathways leads to subacute combined degeneration
Beta chain underproduced - asymptomatic - heterozygote
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

21. What is the aggregation phase of ppf?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Fibrinogen bind GpIIb/IIIa and links platelts
Hereditary elloptocytosis
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

22. What state is commonly associated with nonHod lymphoma
Howell - Jolly bodies - hypo/asplenia
Deficiency in factor VIII
HIV or immunosupression
Sickle cell

23. Adults present with cutaneous patches/nodules - indolent CD4+
Mycosis fundgoides/Sezary syndrome
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
AML
Birbeck granules

24. What is the pattern of involvement and spread for nonHod lympho
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Pos
Inc RBC - dec O2 sat - inc EPO
Blasts > 5%

25. What is the pathogenesis of TTP?
Inc large vWF multimers - inc platelet aggregation and thrombosis
VWF carries/protects factor VIII
MGUS - monoclonal gammopathy of undetermined significance
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

26. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Lymphoma
TPA - cleavage of fibrin mesh
Factor V Leiden
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

27. What is the characteristic finding for MAHA on peripheral smear?
Follicular lymphoma - indolent course
Imatinib - anti bcr abl
Schistocytes - helmet cells
Porphyrias

28. Deficiency in GpIIb/IIIa

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29. Megakaryocytosis
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Essential thrombocytosis
Helminth infections major basic protein

30. Why does B12 def cause neuro sx?
Multiple myeloma
Extravascular
Protein S - cleaves and invactivates Va and VIIIa
B12 in fatty acid pathways leads to subacute combined degeneration

31. mother's antibodies attack fetal RBCs
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Macrohemorrhage - hemarthroses - easy bruising
Acts to stabilize platelet plug

32. hemolytic in a newborn - dec ATP and rigid RBCs
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Hairy cell leukemia
Pyruvate kinase def - extravascular
Beta chain underproduced - asymptomatic - heterozygote

33. What does NAACP stand for - in regards to the causes of eosinophiia?
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Malaria - Babesia
ADP and Ca

34. What do plasma cells do
Porphyrias
Diffuse Large b cell lymphoma
Produce antibody - lots of RER and golgi
Easy gas exchange

35. 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
Anemia of chronic disease - Aplastic anemia - kidney disease
TRAP (tartrate resistant acid phosphatase
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

36. What are the pro aggregation factors?
Intravascular hemolysis
Peroxidase
Burkitt lymphoma
TXA2 - dec blood flow - inc platelet aggregation

37. What are the variants of the RS cells
5- FU - AZT - hydroxyurea
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Gp1b
Lacunar in nodular sclerosing variant

38. What portion of IgE can mast cells bind
B6 therapy (pyrodixine)
Dec
1 to 6
Fc

39. What are the four levels of alpha thal?
Factor V Leiden
Anti Rh
Antigen - antibody
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

40. What percentage of WBCs are eosinophils?
CML to AML or All
Helminth infections major basic protein
Parvovirus
1 to 6

41. Basophilica stippling
Examples of oxidative stress
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

42. Where do you see hypersegmented polys?
B12/folate def
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Tissue factor converst the VII and VIIa
MHC II and Fc receptors

43. Basophilic nuclear remnants fonud in RBCs
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Howell - Jolly bodies - hypo/asplenia
B12 def
VIII

44. What is the pathogenesis of ACD?
Diffuse Large b cell lymphoma
Complications of sickle cell anemia
90% anearobically from glucose to lactate - 10% from HMP shunt
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

45. What does LEAD stand for in lead poisoning?
Adult T cell lymphoma - presents with cutaneous lesions
TPA - cleavage of fibrin mesh
Direct coombs - agglutinate if RBCs are coated with Ig
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 -

46. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
Protein S - cleaves and invactivates Va and VIIIa
Epoxide reductase - warfarin inhibits
DIC

47. Which immunoglobulin is involved in warm agglutination?
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
IgG - Warm is GREAT
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Extrinsic - I - II - V - VII and X

48. What chromosomal translocation is associated with a better prognosis in All
T(12;21)
Hereditary elloptocytosis
All
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

49. What is the receptor for fibrinogen?
Factor V resistant to activated protein C's inhibition
Ticlopidine/clopidogrel
Paroxysmal nocturnal hemoglobinuria
GpIIb/IIIa

50. What is the treatment to prevent a woman from forming anti Rh antibody?
Rhogam - Rh antigen immunoglobulin
Porphobilinogen deaminase aka uroporphyrinogen I synthase
No lytic bone lesions in WM
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

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