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Hemeonc

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Answer 50 questions in 15 minutes.
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1. What is the affected enzyme in lead poisoning
Intravascular hemolysis
HIV or immunosupression
Ferrocheletase and ALA dehydrogenase
Peroxidase

2. What is the enzyme defect in Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Signs and sx of aplastic anemia
Gp1b
CLL (SLL without the peripheral lymphocytosis

3. What does NAACP stand for - in regards to the causes of eosinophiia?
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Fe def - thal - pb poison - sideroblastic anemia
Cromolyn sodium

4. anisocytosis
Acts to stabilize platelet plug
Peroxidase
Varying sizes
B12/folate def

5. What is the pathogenesis of sickle cell
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
VWF carries/protects factor VIII
Howell Jolly bodies
Essential thrombocytosis

6. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Helps platelts adhere to endothelium
SLE - CLL - alpha methyldopa
Hereditary spherocytosis
Porphobilinogen - delta ALA - uroporphyrin

7. What causes the physiologic chloride shift and What does the chloride shift do?
Idiopathic thrombocytopenia (ITP)
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Hodgkin
Beta chain underproduced - asymptomatic - heterozygote

8. What is the main source of energy in RBCs
M3 AML (acute promyelocytic leukemia)
Sideroblastic anemia
GpIIb/IIIa
90% anearobically from glucose to lactate - 10% from HMP shunt

9. Macro - ovalocyte
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Kids= exposure to lead paint - adults = battery - ammunition factory
Blasts > 5%

10. iron deficiency anemia - esophageal web - atrophic glossitis
CML
Blood
Plummer - vinson syndrome
VWD

11. poikilocytosis
Bernard soulier
Tissue
Varying shapes
B antigena and A antibodes

12. What is the effected enzyme in acute intermittment porphyria?
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Blasts > 5%
Induces differentiation of myeloblasts
Porphobilinogen deaminase aka uroporphyrinogen I synthase

13. What virus is associated with Burkitt lymphoma
Anemia of chronic disease - Aplastic anemia - kidney disease
Complications of sickle cell anemia
B cells
EBV

14. Serum iron - transferrin - ferritin lab values for hemochromatosis
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Up - down - up
Paraprotein spike - monoclonal protein
Porphobilinogen - delta ALA - uroporphyrin

15. What is the most common hodgkin lymphoma
Nodular sclerosing
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Positive osmootic fragility test and splenectomy
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

16. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Leukemia
Protein C or S deficiency
B antigena and A antibodes
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

17. Elliptocyte
Folate def
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Hereditary elloptocytosis
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

18. What indicated a good prognosis in Hodgkin lymphoma?
Inc lymphoctes - and less RS cells
Varying shapes
Beta thal
Dendritic cells?

19. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Pyruvate kinase def - extravascular
Leukemoid reaction
Pos
Contiguous

20. Elderly - mature b cell tumor with filamentous - hairlike projections
Factor V resistant to activated protein C's inhibition
Hairy cell leukemia
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
IFN gama

21. What is relative polycythemia
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Dec plasma volume
Pyruvate kinase def - extravascular
Her next Rh+ fetus

22. Serum iron - transferrin - ferritin lab values for pregs - OCP use
A antigen and B antibodies
AB - no antibodies
Nl - up - nl
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

23. Sickle cell
Sickle cell anemia
Contiguous
Inactivates it
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

24. What are the labs and tx for HS?
Def in factor IX
Positive osmootic fragility test and splenectomy
Lymphoma
Inc vasodiltion - inc perm - inc pain

25. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Down - down - up
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
CNS and testis
Intravascular hemolysis

26. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Von Willebrand's disease
AB - no antibodies
Parvovirus
VWD

27. What virus is associated with 50% of Hodgkin lymphoma
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
VWF and fibrinogen
Fibrinogen bind GpIIb/IIIa and links platelts
EBV

28. What percentage of WBCs are eosinophils?
Thrombotic thrombocytopenic purpura
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Orotic aciduria
1 to 6

29. Can B cells function as APCs?
Beta 4
Incactivates II - VII - IX - X - XI - XII
Yes - via MHC II
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

30. What does ADP do?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Helps platelts adhere to endothelium
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Sideroblastic anemia

31. Deficiency in vWF

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32. How is beta thal minor dx
8-10 days
Hodgkin
Inc HbA2 on electrophoresis
Protoporphyrin (blood)

33. What are the neuro sx of B12 def?
Inc HbA2 on electrophoresis
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Splicing sites and promotor sequences

34. What is the therapy for CML (philly chrom)
EBV
Imatinib - anti bcr abl
Acts to stabilize platelet plug
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

35. What is the life span of a normal RBC
Spleen
120 days
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Tissue factor converst the VII and VIIa

36. Which factor does vWF carry/protect
1 to 6
Glucose and heme - which inhibit ALA synthase
VIII
Positive osmootic fragility test and splenectomy

37. CD5+ - poor prognosis - t(11;14)
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Aspirin
Mantle cell - older males
Bleeding - malnutrition/absorption - inc demand pregs

38. HTLV-1
A antigen and B antibodies
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Adult T cell lymphoma - presents with cutaneous lesions
Exposed collagen upon endothelial damage

39. What is the clinical picture of hemophilia A or B
Pyruvate kinase def - extravascular
Bleeding - malnutrition/absorption - inc demand pregs
Macrohemorrhage - hemarthroses - easy bruising
Schistocytes and inc LDH

40. What is the philadelphia chromosome
CML
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
T(9;22) bcr abl
Fibrinogen

41. Upregulated growth of leukocytes in bone marro
Glycine and succinyl - coa
Fibrinogen
Glanzmann's throbmasthenia
Leukemia

42. Blood type B
B antigena and A antibodes
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
VIII

43. universal donor
1 to 6
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Anti Rh
No antigen - both antibodies

44. After an injury - What does vWF bind to begin platelet plug formation?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Paraprotein spike - monoclonal protein
Exposed collagen upon endothelial damage
Eleveated PT - PTT

45. Which cell is neoplastic in multiple myeloma
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Plasma cell
Sheets of lymphocytes interspersed with macs
Dendritic cells?

46. What are the pro aggregation factors?
Mycosis fundgoides/Sezary syndrome
T(9;22) bcr abl
1 to 6
TXA2 - dec blood flow - inc platelet aggregation

47. Who has more severe disease - HbSS or HbSC
Def in factor IX
HbSS
Beta chain underproduced - asymptomatic - heterozygote
Down - down - up

48. Where are basophils found?
Bimodal - men>women - except for nodular sclerosing type
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Blood
All trans retinoic acid

49. Bone pain plus anemia
Multiple myeloma
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Yes - via MHC II
Blod - tissue - MACS

50. What does Vit K deficiency cause?
Helps platelts adhere to endothelium
EBV
Dec synthesis of factors 1972 - protein C/S
Langerhans cells

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