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Hemeonc

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1. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
Leukemia
All
Protoporphyrin (blood)
Up - down - nl

2. hypocellular bone marrown tih fatty infiltration
Complications of sickle cell anemia
Hairy cell leukemia
Protoporphyrin (blood)
Aplastic anemia - pancytopenia

3. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Sideroblastic anemia
Paraprotein spike - monoclonal protein
SLE - CLL - alpha methyldopa
Sheets of lymphocytes interspersed with macs

4. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
A antigen and B antibodies
Blood
ATIII def
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

5. Fibrotic obliteration of bone marow with teardrop cells

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6. How are plasma cells characterized?
Low in CML
Off center nuclues - clock face chromatin
No platelet clumping
Sideroblastic anemia

7. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
VIII
Intravascular hemolysis
Sheets of lymphocytes interspersed with macs
Produce antibody - lots of RER and golgi

8. What do platelets interact with to form a hemostatic plug
PGI2 - NO inc blood flow - dec platelet aggregation
No antigen - both antibodies
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 -
Fibrinogen

9. 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
Inducers of primary antibody response
Sickle cell
Dendritic cells?
Both alpha and beta thal

10. Deficiency in GpIIb/IIIa

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11. What are the neuro sx of B12 def?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
No
Exposed collagen upon endothelial damage
Langerhans cells

12. What virus can cause an aplastic crisis in pts with HS?
VWD
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Burkitt lymphoma
Parvovirus

13. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
IFN gama
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Up - down - nl
Decrease EPO

14. Upregulated growth of leukocytes in bone marro
Leukemia
CNS and testis
1 to 6
Langerhans cell histiocytosis

15. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Nl - up - nl
Glanzmann's throbmasthenia
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Lysine for glutamate at position 6

16. What is the effected enzyme in acute intermittment porphyria?
Down - up - down
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

17. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Direct coombs - agglutinate if RBCs are coated with Ig
Follicular lymphoma - indolent course
Low in CML

18. What begins the extrinsic pathway?
Fibrinogen
Tissue factor converst the VII and VIIa
Signs and sx of aplastic anemia
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

19. What is their role?
Low O2 in papilla; can also get microhematuria from medullary infarcts
8-10 days
Inducers of primary antibody response
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

20. What is the tx for vWD
AML
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Bleeding - malnutrition/absorption - inc demand pregs
DIC - TTP/HUS - traumatic hemolysis

21. Conditions of defective heme synthesis leading to accumulation of heme precurors
Leukemoid reaction
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Porphyrias
DIC

22. Megakaryocytosis
B12/folate def
20 to 40
Off center nuclues - clock face chromatin
Essential thrombocytosis

23. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Fibrinogen bind GpIIb/IIIa and links platelts
CLL (SLL without the peripheral lymphocytosis
Low in CML
Induces differentiation of myeloblasts

24. What does the blood smear show in glanzmann's?
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Beta chain underproduced - asymptomatic - heterozygote
GpIIb/IIIa
No platelet clumping

25. What is hemophiliia A
Fe def - thal - pb poison - sideroblastic anemia
Beta chain underproduced - asymptomatic - heterozygote
Deficiency in factor VIII
Plummer - vinson syndrome

26. What are the variants of the RS cells
Inc UCB
Round densly staining nucleus with a small amount of pale cytoplasm
Lacunar in nodular sclerosing variant
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

27. What is the characteristic histologic finding in Hodgkin Lymphoma
Reed - sternberg cells
M3 AML (acute promyelocytic leukemia)
B symptoms - fever night sweats - weight loss
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

28. What is the pathogenesis of TTP?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Inc large vWF multimers - inc platelet aggregation and thrombosis
Ticlopidine/clopidogrel
Prothrombin gene mutation

29. What substances are released in mast cell degranulation
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Histamine - heparin - and eosinohil chemotactic factors
Sheets of lymphocytes interspersed with macs
Deficiency in factor VIII

30. What is the therapy for the M3 variant?
B6 therapy (pyrodixine)
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
All trans retinoic acid
Easy gas exchange

31. What vitamin is a cofactor for the first step of heme synthesis
Mantle cell lymphoma
B6
No antigen - both antibodies
Hereditary spherocytosis - autoimmune hemolysis

32. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Cromolyn sodium
Sideroblastic anemia
Polycythemia vera
Inactivates it

33. t(11;14)
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Mantle cell lymphoma
MGUS - monoclonal gammopathy of undetermined significance

34. What is the mutation in HbS
Lysine for glutamate at position 6
Blasts > 5%
Valine for glutamate
Blistering cutaneous photosens - most common porphyria

35. Serum iron - transferrin - ferritin lab values for iron def anemia
All trans retinoic acid
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Induces differentiation of myeloblasts
Down - up - down

36. Basophilic nuclear remnants fonud in RBCs
Parvovirus
Glucose and heme - which inhibit ALA synthase
M3 AML (acute promyelocytic leukemia)
Howell - Jolly bodies - hypo/asplenia

37. Causes of iron def?
Low in CML
Dendritic cells?
Varying sizes
Bleeding - malnutrition/absorption - inc demand pregs

38. What is the treatment for acute intermittent porphyria
Glucose and heme - which inhibit ALA synthase
Indirect coombs - agglutinate if serum anti RBC surface Ig
Macrohemorrhage - hemarthroses - easy bruising
Heinz bodies - seen in alpha thal and G6PD

39. What is the coombs test results in AIHA?
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Pos
TXA2 - dec blood flow - inc platelet aggregation

40. What is the age breakdown for hodgkins
Bimodal - men>women - except for nodular sclerosing type
Thrombotic thrombocytopenic purpura
Adult T cell lymphoma - presents with cutaneous lesions
Ferrocheletase and ALA dehydrogenase

41. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Nl PT - elevated PTT - intrinsic pathway defect
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
G6PD

42. How does vWD cause elevated PTT?
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Decrease EPO
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
VWF carries/protects factor VIII

43. What is factor V leidin?

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44. What finding you do you see in patients after splenectomy
Yes - via MHC II
Howell Jolly bodies
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Bone marrow infiltration - myelofibrosis

45. HTLV-1
PGI2 - NO inc blood flow - dec platelet aggregation
Blistering cutaneous photosens - most common porphyria
Epoxide reductase - warfarin inhibits
Adult T cell lymphoma - presents with cutaneous lesions

46. What does hairy cell leukemia stain with
Her next Rh+ fetus
TRAP (tartrate resistant acid phosphatase
Lung disease - congenital heart diseaes - and high altitude
Leukemoid reaction

47. What do eosinophils defend against and What do they use to do it?
IFN gama
Lysosomes
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Helminth infections major basic protein

48. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Off center nuclues - clock face chromatin
Complications of sickle cell anemia
Hodgkin

49. What is the enzyme defect in Porphyria cutanea tarda
Indirect coombs - agglutinate if serum anti RBC surface Ig
40 to 75
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Uroporphyrinogen decarboxylase

50. Where are mast cells found
Anemia of chronic disease - Aplastic anemia - kidney disease
Sickle cell anemia
Nl - up - nl
Tissue

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Hemeonc

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