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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 effected enzyme in acute intermittment porphyria?
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
ADP and Ca
Porphobilinogen deaminase aka uroporphyrinogen I synthase
DDAVP (desmopressin) which releases stored vWF stored in endothelium

2. What is the main source of energy in RBCs
90% anearobically from glucose to lactate - 10% from HMP shunt
Sideroblastic anemia
Signs and sx of aplastic anemia
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

3. Serum iron - transferrin - ferritin lab values for hemochromatosis
Ewing sarcoma
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Up - down - up
Histaminase and arylsulfatase

4. What is the pathogenesis of sickle cell
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
1 to 6
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Ferrocheletase and ALA dehydrogenase

5. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Low in CML
Ringed sideronblasts with iron laden mitochondria
40 to 75
Inc

6. What virus can cause an aplastic crisis in pts with HS?
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Intrinsic - all factors except - VII - XIII
Parvovirus
Antigen - antibody

7. What is the accumulated substance in lead poisoning
Bone marrow infiltration - myelofibrosis
Lysosomes
CML
Protoporphyrin (blood)

8. Blood type A
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
A antigen and B antibodies
M3 AML (acute promyelocytic leukemia)
Up - down - up

9. Ddx for microcytic anemia
Bite cells and Heinz bodies
Fe def - thal - pb poison - sideroblastic anemia
Her next Rh+ fetus
Ringed sideronblasts with iron laden mitochondria

10. What substance accumulates in porphyria cutanea
Uroporphyrin (tea colored urine)
Abciximab
Protein S - cleaves and invactivates Va and VIIIa
Inc vasodiltion - inc perm - inc pain

11. What is the treatment for lead poising?
2 to 10
TRAP (tartrate resistant acid phosphatase
Bimodal - men>women - except for nodular sclerosing type
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

12. Bite cell
IFN gama
PGI2 - NO inc blood flow - dec platelet aggregation
G6PD
Helminth infections major basic protein

13. Back pain - hemoglobinuria
Common a few days after oxidative stress in a pt with G6PD
Beta thal
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Lead - and EtOH

14. After an injury - What does vWF bind to begin platelet plug formation?
Myelofibrosis (marow is crying being its fibrosed'
Factor V resistant to activated protein C's inhibition
Leukemia
Exposed collagen upon endothelial damage

15. Acanthocyte (spur cell)
Inducers of primary antibody response
Abciximab
Liver disease - abetalipoproteinemia - acntho = spiny
SLE - CLL - alpha methyldopa

16. What do you see in peripheral smear in a pt with G6PD?
Megakaryocytes
Bite cells and Heinz bodies
Nl - up - nl
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

17. What are the pro aggregation factors?
TXA2 - dec blood flow - inc platelet aggregation
EBV
Histamine - heparin - and eosinohil chemotactic factors
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

18. mother's antibodies attack fetal RBCs
Plasma cell
Positive osmootic fragility test and splenectomy
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Varying sizes

19. What the alpha granules contain in platelets?
Polycythemia vera
Nl PT - elevated PTT - intrinsic pathway defect
Lung disease - congenital heart diseaes - and high altitude
VWF and fibrinogen

20. What do the iron studies show in sideroblastic anemia
Nl PT - elevated PTT - intrinsic pathway defect
Signs and sx of aplastic anemia
Liver disease - abetalipoproteinemia - acntho = spiny
Inc serum iron - normal TIBC - inc ferratin

21. Drug that inhibits COX and therefore TXA2 synthesis
No
Aspirin
DIC
B12 def

22. Which cell is neoplastic in multiple myeloma
Peroxidase
Degrades fibrin mesh and converts C3 to C3a
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Plasma cell

23. Mutation in 3' untranslated region associated with venous clots
VWD
Prothrombin gene mutation
MGUS - monoclonal gammopathy of undetermined significance
Extravascular

24. Upregulated growth of leukocytes in bone marro
Leukemia
Anti Rh
Histamine - heparin - and eosinohil chemotactic factors
EBV

25. What cell is primarily involved in non Hod lymph
B cells
B12 in fatty acid pathways leads to subacute combined degeneration
Glycine and succinyl - coa
Inc lymphoctes - and less RS cells

26. Where does All spread
CNS and testis
Petechiae
Induces differentiation of myeloblasts
Bimodal - men>women - except for nodular sclerosing type

27. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Hereditary spherocytosis
Langerhans cells
ADP and Ca
Down - down - up

28. What are the etiologies of B12 def
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Folate def
Helminth infections major basic protein
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

29. What is the receptor for fibrinogen?
Nodular sclerosing
GpIIb/IIIa
Blod - tissue - MACS
Complications of sickle cell anemia

30. What is a metabolic disorder tht can cause macrocytic anemia?
Orotic aciduria
Howell - Jolly bodies - hypo/asplenia
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Fc

31. What do plasma cells do
Helps platelts adhere to endothelium
Produce antibody - lots of RER and golgi
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

32. What are dendritic cells called in the skin?
Inc HbF and dec HbS
Sheets of lymphocytes interspersed with macs
Indirect coombs - agglutinate if serum anti RBC surface Ig
Langerhans cells

33. What is the age breakdown for hodgkins
TRAP (tartrate resistant acid phosphatase
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
No lytic bone lesions in WM
Bimodal - men>women - except for nodular sclerosing type

34. Target cell

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35. What is the are the presenting symptoms of lead poisoning in kids and adults
1 to 6
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
ADP and Ca
EBV

36. Is G6PD intravascular or extravascular
Intravascular
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Ankryin - band 4.1 or spectrin
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

37. What does bradykinin do?
Up - down - up
2 to 10
AB - no antibodies
Inc vasodiltion - inc perm - inc pain

38. What is the characteristic histologic finding in Hodgkin Lymphoma
Adult T cell lymphoma - presents with cutaneous lesions
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Inc serum iron - normal TIBC - inc ferratin
Reed - sternberg cells

39. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
HIV or immunosupression
Beta thal
VWD
Blod - tissue - MACS

40. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Dendritic cells?
Factor V Leiden
Pyruvate kinase def - extravascular
G6PD

41. What is HbH
Ferrocheletase and ALA dehydrogenase
Beta 4
Hereditary elloptocytosis
CNS and testis

42. What is the pathogenesis of TTP?
Inc large vWF multimers - inc platelet aggregation and thrombosis
Hereditary spherocytosis - autoimmune hemolysis
Blistering cutaneous photosens - most common porphyria
B12/folate def

43. What is the general pathology of a macrocytic anemia?
Diffuse Large b cell lymphoma
Produce antibody - lots of RER and golgi
Lysine for glutamate at position 6
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

44. What converts plasminogen to plasm and What does plasmin do?
Signs and sx of aplastic anemia
Positive osmootic fragility test and splenectomy
TPA - cleavage of fibrin mesh
Fe def - thal - pb poison - sideroblastic anemia

45. What is the presenting scenario for TTP?
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
DDAVP (desmopressin) which releases stored vWF stored in endothelium
CML
Complications of sickle cell anemia

46. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
8-10 days
Hereditary spherocytosis - autoimmune hemolysis
CNS and testis
Intravascular hemolysis

47. What percentage if WBCs are polys?
Protoporphyrin
40 to 75
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Thrombotic thrombocytopenic purpura

48. What is the difference of presentation of Burkitt in Africa vs the United States
Sideroblastic anemia
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Africa = Jaw lesion - US = pelvis or abdomen
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

49. What finding you do you see in patients after splenectomy
Low O2 in papilla; can also get microhematuria from medullary infarcts
Howell Jolly bodies
VWF carries/protects factor VIII
Eleveated PT - PTT

50. What are the etiologies of folate def
No
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Liver disease - abetalipoproteinemia - acntho = spiny
Leukemia