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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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This is a study tool. The 3 wrong answers for each question are randomly chosen from answers to other questions. So, you might find at times the answers obvious, but you will see it re-enforces your understanding as you take the test each time.

1. What is the treatment for acute intermittent porphyria
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Glucose and heme - which inhibit ALA synthase
Kids= exposure to lead paint - adults = battery - ammunition factory

2. What are the pro aggregation factors?
B antigena and A antibodes
Inc vasodiltion - inc perm - inc pain
IFN gama
TXA2 - dec blood flow - inc platelet aggregation

3. What is appropriate absolyte polycythemia associated with
5- FU - AZT - hydroxyurea
Lung disease - congenital heart diseaes - and high altitude
ADP and Ca
Fibrinogen

4. What is appropriate absolute polycythemia
Inc RBC - dec O2 sat - inc EPO
CLL (SLL without the peripheral lymphocytosis
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Tissue factor converst the VII and VIIa

5. What does ectopic EPO produce
Langerhans cells
Glucose and heme - which inhibit ALA synthase
Lung disease - congenital heart diseaes - and high altitude
Inappropriate absolute with inc RBCs and EPO

6. What is the defect in beta thal?
Fibrinogen bind GpIIb/IIIa and links platelts
MGUS - monoclonal gammopathy of undetermined significance
CML to AML or All
Splicing sites and promotor sequences

7. Megakaryocytosis
TRAP (tartrate resistant acid phosphatase
Protoporphyrin (blood)
Multiple myeloma
Essential thrombocytosis

8. Wilm's tumor - RCC - HCC - hydronephrosis
20 to 40
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Blasts > 5%

9. What is HbH
Blod - tissue - MACS
Beta 4
Polycythemia vera
Prothrombin gene mutation

10. mother's antibodies attack fetal RBCs
EBV
Low O2 in papilla; can also get microhematuria from medullary infarcts
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
DIC

11. What causes hydrops fetalis
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Hb Barts - gamma4 in defect in all 4 alpha genes
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
VWF carries/protects factor VIII

12. What is the main source of energy in RBCs
Essential thrombocytosis
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Protein C or S deficiency
90% anearobically from glucose to lactate - 10% from HMP shunt

13. What does increasing heme do to ALA synthase activity
Howell - Jolly bodies - hypo/asplenia
Polycythemia vera
IgG - Warm is GREAT
Dec

14. Drug that inhibits COX and therefore TXA2 synthesis
Aspirin
No platelet clumping
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Petechiae

15. What is the tx for aplastic anemia
Nl PT - elevated PTT - intrinsic pathway defect
DIC
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Abciximab

16. What is the affected enzyme in lead poisoning
Ferrocheletase and ALA dehydrogenase
Histaminase and arylsulfatase
Liver disease - abetalipoproteinemia - acntho = spiny
120 days

17. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Glanzmann's throbmasthenia
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Hereditary spherocytosis

18. What are the presenting symptoms of porphyria cutanea tarda
Blistering cutaneous photosens - most common porphyria
Reed - sternberg cells
Fe def - thal - pb poison - sideroblastic anemia
CNS and testis

19. Where do you see hypersegmented polys?
Degrades fibrin mesh and converts C3 to C3a
SLE - CLL - alpha methyldopa
Multiple myeloma
B12/folate def

20. Back pain - hemoglobinuria
Extrinsic - I - II - V - VII and X
Common a few days after oxidative stress in a pt with G6PD
Extravascular hemolysis
Ticlopidine/clopidogrel

21. What does hairy cell leukemia stain with
ADP and Ca
MGUS - monoclonal gammopathy of undetermined significance
TRAP (tartrate resistant acid phosphatase
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 -

22. What is the coombs test results in AIHA?
Megakaryocytes
Leukemia
B symptoms - fever night sweats - weight loss
Pos

23. What are the azuraphilic granules in PMNs
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Lysosomes
Africa = Jaw lesion - US = pelvis or abdomen
Rhogam - Rh antigen immunoglobulin

24. What virus is associated with Burkitt lymphoma
Indirect coombs - agglutinate if serum anti RBC surface Ig
EBV
Macrohemorrhage - hemarthroses - easy bruising
Inc UCB

25. Spherocyte
Bernard soulier
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Hereditary spherocytosis - autoimmune hemolysis
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

26. What do the iron studies show in sideroblastic anemia
Paraprotein spike - monoclonal protein
Glucose and heme - which inhibit ALA synthase
Produce antibody - lots of RER and golgi
Inc serum iron - normal TIBC - inc ferratin

27. What percentage of WBCs are eosinophils?
1 to 6
B symptoms - fever night sweats - weight loss
Bimodal - men>women - except for nodular sclerosing type
EBV

28. How does vWD cause elevated PTT?
Glucose and heme - which inhibit ALA synthase
VIII
VWF carries/protects factor VIII
Bone marrow - thymus - blood (80% of circulating lymphos are T)

29. What vitamin is a cofactor for the first step of heme synthesis
B6
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
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 -
Intravascular

30. Why does B12 def cause neuro sx?
B12 in fatty acid pathways leads to subacute combined degeneration
CLL (SLL without the peripheral lymphocytosis
Pyruvate kinase def - extravascular
Both alpha and beta thal

31. How are plasma cells characterized?
Off center nuclues - clock face chromatin
Fibrinogen
Plasma cell
Dec

32. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Folate def
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Down - down - up

33. What CD molecules are on RS cells
Extrinsic - I - II - V - VII and X
CD15 and CD30 pos
M3 AML (acute promyelocytic leukemia)
Ticlopidine/clopidogrel

34. Which cell is neoplastic in multiple myeloma
Essential thrombocytosis
Nodular sclerosing
Plasma cell
Leukemia

35. Where are monocytes typically found - where do they go - and What do the differentiate into?
Decrease EPO
Blod - tissue - MACS
Examples of oxidative stress
TXA2 - dec blood flow - inc platelet aggregation

36. Teardrop cell
Collagen - BM - activated platelets
Bone marrow infiltration - myelofibrosis
Follicular lymphoma - indolent course
Bite cells and Heinz bodies

37. Who are the professional APCs?
Dendritic cells?
Hereditary spherocytosis - autoimmune hemolysis
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Bleeding - malnutrition/absorption - inc demand pregs

38. Adults present with cutaneous patches/nodules - indolent CD4+
Helps platelts adhere to endothelium
SLE - CLL - alpha methyldopa
Mycosis fundgoides/Sezary syndrome
Inc serum iron - normal TIBC - inc ferratin

39. What is the enzyme defect in Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Hodgkin
Common a few days after oxidative stress in a pt with G6PD
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

40. Ddx for microcytic anemia
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
B symptoms - fever night sweats - weight loss
Extravascular
Fe def - thal - pb poison - sideroblastic anemia

41. anisocytosis
Varying sizes
B symptoms - fever night sweats - weight loss
90% anearobically from glucose to lactate - 10% from HMP shunt
Inc large vWF multimers - inc platelet aggregation and thrombosis

42. What is the effect of ACE on bradykinin
Aspirin
Inactivates it
Low O2 in papilla; can also get microhematuria from medullary infarcts
G6PD

43. What substance prevents mast cells degranulation?
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Cromolyn sodium
Decrease EPO
Positive osmootic fragility test and splenectomy

44. 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
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
G6PD
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

45. What is hemophiliia A
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Deficiency in factor VIII
EBV
Parvovirus

46. What is the most common hodgkin lymphoma
Glanzmann's throbmasthenia
Paroxysmal nocturnal hemoglobinuria
Mantle cell lymphoma
Nodular sclerosing

47. What substance is Fe added to to yield heme
Inc HbF and dec HbS
Protoporphyrin
Hairy cell leukemia
Alpha thal - asian and african american

48. Who has more severe disease - HbSS or HbSC
Anti Rh
HbSS
Tissue
Complications of sickle cell anemia

49. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Tissue
Hereditary elloptocytosis
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Signs and sx of aplastic anemia

50. Plasma cell neoplasm
Multiple myeloma
CD15 and CD30 pos
Inc HbA2 on electrophoresis
Langerhans cell histiocytosis