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Hemeonc

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1. What is appropriate absolyte polycythemia associated with
T(12;21)
Valine for glutamate
Lung disease - congenital heart diseaes - and high altitude
IFN gama

2. Which pathway and factorrs are tested by the PT coag test
Intravascular
Extrinsic - I - II - V - VII and X
Leukemia
PGI2 - NO inc blood flow - dec platelet aggregation

3. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
Anti Rh
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Def in factor IX
No lytic bone lesions in WM

4. How is beta thal minor dx
Plasma cell
Inc HbA2 on electrophoresis
Varying sizes
B symptoms - fever night sweats - weight loss

5. Adults present with cutaneous patches/nodules - indolent CD4+
Mycosis fundgoides/Sezary syndrome
T(12;21)
Liver disease - abetalipoproteinemia - acntho = spiny
Fibrinogen bind GpIIb/IIIa and links platelts

6. iron deficiency anemia - esophageal web - atrophic glossitis
IFN gama
Low in CML
Epoxide reductase - warfarin inhibits
Plummer - vinson syndrome

7. Conditions of defective heme synthesis leading to accumulation of heme precurors
Extravascular
Produce antibody - lots of RER and golgi
Porphyrias
Parvovirus

8. What is the pathogenesis of aplastic anemia with kidney disease
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Hereditary elloptocytosis
Splicing sites and promotor sequences
Decrease EPO

9. mother's antibodies attack fetal RBCs
Dec
Inc HbF and dec HbS
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Low O2 in papilla; can also get microhematuria from medullary infarcts

10. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Heinz bodies - seen in alpha thal and G6PD
Multiple myeloma
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

11. What is the treatment for sideroblastic anemia
B6 therapy (pyrodixine)
Extravascular hemolysis
M3 AML (acute promyelocytic leukemia)
Diffuse Large b cell lymphoma

12. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
ATIII def
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Cromolyn sodium
G6PD

13. What is the aggregation phase of ppf?
A antigen and B antibodies
Bite cells and Heinz bodies
Abciximab
Fibrinogen bind GpIIb/IIIa and links platelts

14. What is the characteristic finding for MAHA on peripheral smear?
Heinz bodies - seen in alpha thal and G6PD
Schistocytes - helmet cells
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
CLL (SLL without the peripheral lymphocytosis

15. What are some classic examples of extravascular hemolysis
Kids= exposure to lead paint - adults = battery - ammunition factory
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Hereditary spherocytosis - G6PD - sickle cell
Inc

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)
Beta chain underproduced - asymptomatic - heterozygote
Dec
Complications of sickle cell anemia

17. What are some causes of warm agglutinin autoimmune hemolytic anemia?
SLE - CLL - alpha methyldopa
B6
No antigen - both antibodies
Bone marrow infiltration - myelofibrosis

18. inc LDH - jaundice
Extravascular hemolysis
B antigena and A antibodes
Inactivates it
Imatinib - anti bcr abl

19. Mutation in 3' untranslated region associated with venous clots
Beta 4
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Epoxide reductase - warfarin inhibits
Prothrombin gene mutation

20. What is the characteristic spread of Hodgkin Lymphoma
Contiguous
Schistocytes - helmet cells
B12 in fatty acid pathways leads to subacute combined degeneration
Mycosis fundgoides/Sezary syndrome

21. Fibrotic obliteration of bone marow with teardrop cells

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22. What do you see in vit K def
Bone marrow - thymus - blood (80% of circulating lymphos are T)
B12 def
Eleveated PT - PTT
Folate def

23. Megakaryocytosis
Parvovirus
Mycosis fundgoides/Sezary syndrome
HIV or immunosupression
Essential thrombocytosis

24. When do you see MAHA?
Protoporphyrin (blood)
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Dec synthesis of factors 1972 - protein C/S
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

25. Ringed sideroblasts
Nodular sclerosing
Lysine for glutamate at position 6
Sideroblastic anemia
Hereditary spherocytosis

26. Deficiency in GpIb
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
1 to 6
Bernard soulier
Reed - sternberg cells

27. Basophilica stippling
Leukemia
Her next Rh+ fetus
Sideroblastic anemia
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

28. adults - auer rods - inc circulating myeblasts on peripheral smear
AML
Bernard soulier
CML to AML or All
Ringed sideronblasts with iron laden mitochondria

29. Which pathway and factors are tested in the PTT test
B symptoms - fever night sweats - weight loss
Intrinsic - all factors except - VII - XIII
Up - down - up
Inc HbA2 on electrophoresis

30. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
B12 in fatty acid pathways leads to subacute combined degeneration
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Leukemoid reaction
Extravascular

31. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Indirect coombs - agglutinate if serum anti RBC surface Ig
Factor V Leiden
Orotic aciduria

32. Defect in platelet to platelet adhesion - receptor - disorder - platelet count and bleeding time

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33. Serum iron - transferrin - ferritin lab values for hemochromatosis
Nl PT - elevated PTT - intrinsic pathway defect
Fc
Up - down - up
EBV

34. What is the Ddx for for a macrocytic anemia
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Dec plasma volume
Peroxidase
Sickle cell

35. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Down - down - up
B symptoms - fever night sweats - weight loss
Liver disease - abetalipoproteinemia - acntho = spiny
B cells

36. What causes the physiologic chloride shift and What does the chloride shift do?
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Bite cells and Heinz bodies
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
2 to 10

37. What is the pattern of involvement and spread for nonHod lympho
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Leukemoid reaction
Alpha thal - asian and african american
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

38. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
They bind vWF via GpIb
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Sickle cell

39. Does CML have a JAK2 mut
Adult T cell lymphoma - presents with cutaneous lesions
Lacunar in nodular sclerosing variant
Sickle cell
No

40. What begins the extrinsic pathway?
Glucose and heme - which inhibit ALA synthase
Burkitt lymphoma
Tissue factor converst the VII and VIIa
Multiple myeloma

41. What do you see a starry sky appearance in Burkitt
Mycosis fundgoides/Sezary syndrome
Fibrinogen bind GpIIb/IIIa and links platelts
Sheets of lymphocytes interspersed with macs
Down - up - down

42. Where are 1/3 of platelets stored
Dec
B6
Spleen
Hairy cell leukemia

43. What is contained within the azurophilic granules of PMNs
Africa = Jaw lesion - US = pelvis or abdomen
Protoporphyrin
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Low O2 in papilla; can also get microhematuria from medullary infarcts

44. What is the treatment for acute intermittent porphyria
T(12;21)
Glucose and heme - which inhibit ALA synthase
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

45. What does LEAD stand for in lead poisoning?
TPA - cleavage of fibrin mesh
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
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 -
Lung disease - congenital heart diseaes - and high altitude

46. Where do you see hypersegmented polys?
Lung disease - congenital heart diseaes - and high altitude
B12/folate def
Up - down - up
Extravascular

47. What do the iron studies show in sideroblastic anemia
Inc serum iron - normal TIBC - inc ferratin
VWD
Hairy cell leukemia
Dec synthesis of factors 1972 - protein C/S

48. Where to T cells arise and mature - and migrate to...
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Signs and sx of aplastic anemia
Low in CML
TXA2 - dec blood flow - inc platelet aggregation

49. What is HbH
Beta 4
Adult T cell lymphoma - presents with cutaneous lesions
Dendritic cells?
Complications of sickle cell anemia

50. What is the enzyme defect in Porphyria cutanea tarda
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Her next Rh+ fetus
Uroporphyrinogen decarboxylase
Inc vasodiltion - inc perm - inc pain

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Hemeonc

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