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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 do labs show in DIC?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Schistocytes - helmet cells
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Multiple myeloma

2. What causes renal papillary necrosis in sickle cell
Inc serum iron - normal TIBC - inc ferratin
Howell - Jolly bodies - hypo/asplenia
Low O2 in papilla; can also get microhematuria from medullary infarcts
Prothrombin gene mutation

3. What are the likely exposures of kids and adults for lead poisoning
Sickle cell
Common a few days after oxidative stress in a pt with G6PD
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Kids= exposure to lead paint - adults = battery - ammunition factory

4. What is hemophilia B?
Def in factor IX
Howell - Jolly bodies - hypo/asplenia
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Inc serum iron - normal TIBC - inc ferratin

5. What is the general pathology of a macrocytic anemia?
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
2 to 10
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
MGUS - monoclonal gammopathy of undetermined significance

6. What percentage of WBCs are eosinophils?
1 to 6
Uroporphyrin (tea colored urine)
Rhogam - Rh antigen immunoglobulin
GpIIb/IIIa

7. Which pathway and factors are tested in the PTT test
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Rhogam - Rh antigen immunoglobulin
Incactivates II - VII - IX - X - XI - XII
Intrinsic - all factors except - VII - XIII

8. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Histamine - heparin - and eosinohil chemotactic factors
Incactivates II - VII - IX - X - XI - XII
Birbeck granules

9. what proteins can be defective in HS?
SLE - CLL - alpha methyldopa
Blasts > 5%
8-10 days
Ankryin - band 4.1 or spectrin

10. What cell is primarily involved in non Hod lymph
B cells
Dec
Orotic aciduria
Mycosis fundgoides/Sezary syndrome

11. What is the receptor for fibrinogen?
Inc RBC - dec O2 sat - inc EPO
Langerhans cell histiocytosis
GpIIb/IIIa
Extrinsic - I - II - V - VII and X

12. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
Liver disease - abetalipoproteinemia - acntho = spiny
Ferrocheletase and ALA dehydrogenase
Inc vasodiltion - inc perm - inc pain
All

13. What are the presenting symptoms of acute intermittent porphyria
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Beta thal
Pyruvate kinase def - extravascular
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

14. How are plasma cells characterized?
Off center nuclues - clock face chromatin
Sideroblastic anemia
Inc large vWF multimers - inc platelet aggregation and thrombosis
Extravascular hemolysis

15. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Cromolyn sodium
Pos
Paraprotein spike - monoclonal protein

16. What do the labs show for TTP?
Polycythemia vera
Schistocytes and inc LDH
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

17. Which pathway and factorrs are tested by the PT coag test
Extrinsic - I - II - V - VII and X
T(12;21)
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Incactivates II - VII - IX - X - XI - XII

18. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
VWF carries/protects factor VIII
Dendritic cells?
Down - down - up
Schistocytes and inc LDH

19. anti - Ig antibody added to patients RBCs;
GpIIb/IIIa
Direct coombs - agglutinate if RBCs are coated with Ig
Megakaryocytes
DIC - TTP/HUS - traumatic hemolysis

20. What does the blood smear show in glanzmann's?
Positive osmootic fragility test and splenectomy
Aspirin
Easy gas exchange
No platelet clumping

21. What substance prevents mast cells degranulation?
Cromolyn sodium
Parvovirus
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Diffuse Large b cell lymphoma

22. Serum iron - transferrin - ferritin lab values for iron def anemia
Liver disease - abetalipoproteinemia - acntho = spiny
Low in CML
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 -
Down - up - down

23. Fava beans - sufla drugs - infectinos
Histamine - heparin - and eosinohil chemotactic factors
Her next Rh+ fetus
Varying sizes
Examples of oxidative stress

24. Serum iron - transferrin - ferritin lab values for hemochromatosis
IFN gama
MAHA
Extravascular
Up - down - up

25. Teardrop cell
A antigen and B antibodies
Prothrombin gene mutation
Bone marrow infiltration - myelofibrosis
Down - down - up

26. Which infections can cause MAHA?
Fibrinogen bind GpIIb/IIIa and links platelts
Malaria - Babesia
DIC
Thrombotic thrombocytopenic purpura

27. What do platelets release necessary for coagulation cascaed?
ADP and Ca
90% anearobically from glucose to lactate - 10% from HMP shunt
2 to 10
Helps platelts adhere to endothelium

28. Can B cells function as APCs?
Yes - via MHC II
Extravascular hemolysis
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Paroxysmal nocturnal hemoglobinuria

29. What does ADP do?
T(12;21)
Hereditary spherocytosis
AB - no antibodies
Helps platelts adhere to endothelium

30. How does the therapy for M3 vairant work?
Fc
Induces differentiation of myeloblasts
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Protoporphyrin

31. What is the tx for sickle cell
Hydroxyurea - bone marrow transplant
MAHA
Beta chain underproduced - asymptomatic - heterozygote
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

32. What is the characteristic finding for MAHA on peripheral smear?
Tissue
Schistocytes - helmet cells
120 days
DIC

33. What does STOP Making New Thrombi stand for
Up - down - up
T(12;21)
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Hb Barts - gamma4 in defect in all 4 alpha genes

34. How do platelet disorders present?
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Hereditary spherocytosis - G6PD - sickle cell

35. What are the four levels of alpha thal?
T(12;21)
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
No lytic bone lesions in WM
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

36. What are the labs and tx for HS?
Positive osmootic fragility test and splenectomy
B6 therapy (pyrodixine)
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

37. What is their role?
Myelofibrosis (marow is crying being its fibrosed'
Incactivates II - VII - IX - X - XI - XII
Inducers of primary antibody response
Macrohemorrhage - hemarthroses - easy bruising

38. What is the affected enzyme in acute intermittment porphyria
Blod - tissue - MACS
Inc RBC - dec O2 sat - inc EPO
Porphobilinogen - delta ALA - uroporphyrin
Leukemoid reaction

39. What activates the intrinsic pathway?
Common a few days after oxidative stress in a pt with G6PD
Glycine and succinyl - coa
2 to 10
Collagen - BM - activated platelets

40. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Alpha thal - asian and african american
Bone marrow infiltration - myelofibrosis
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Factor V resistant to activated protein C's inhibition

41. Acanthocyte (spur cell)
Up - down - up
Inc HbA2 on electrophoresis
CD15 and CD30 pos
Liver disease - abetalipoproteinemia - acntho = spiny

42. What does ectopic EPO produce
Induces differentiation of myeloblasts
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Inappropriate absolute with inc RBCs and EPO
Beta 4

43. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Nodular sclerosing
Mycosis fundgoides/Sezary syndrome
HIV or immunosupression
Intravascular hemolysis

44. t(8;14) c - myc gene
Burkitt lymphoma
Varying shapes
Indirect coombs - agglutinate if serum anti RBC surface Ig
90% anearobically from glucose to lactate - 10% from HMP shunt

45. When do you see MAHA?
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Protoporphyrin (blood)
Anti Rh
B12 def

46. What is the most common nonhodgkin lymphoma
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Inc HbF and dec HbS
Up - down - nl
Diffuse Large b cell lymphoma

47. hypocellular bone marrown tih fatty infiltration
Aplastic anemia - pancytopenia
Nl - up - nl
...
No platelet clumping

48. What is the pathogenesis of TTP?
Inc large vWF multimers - inc platelet aggregation and thrombosis
Collagen - BM - activated platelets
Langerhans cell histiocytosis
Hb Barts - gamma4 in defect in all 4 alpha genes

49. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Nl PT - elevated PTT - intrinsic pathway defect
Dec plasma volume
CLL (SLL without the peripheral lymphocytosis
Peroxidase

50. Mutation in 3' untranslated region associated with venous clots
40 to 50
Up - down - up
Howell Jolly bodies
Prothrombin gene mutation