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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 pathogenesis of sickle cell
Pos
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Hairy cell leukemia

2. What are some classic examples of extravascular hemolysis
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Hereditary spherocytosis - G6PD - sickle cell
CLL (SLL without the peripheral lymphocytosis
Idiopathic thrombocytopenia (ITP)

3. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
All
Schistocytes - helmet cells
Inc RBC - dec O2 sat - inc EPO
Intrinsic - all factors except - VII - XIII

4. What finding you do you see in patients after splenectomy
Gp1b
Howell Jolly bodies
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Burkitt lymphoma

5. What indicated a good prognosis in Hodgkin lymphoma?
Inc lymphoctes - and less RS cells
1 to 6
Direct coombs - agglutinate if RBCs are coated with Ig
Inc RBC - dec O2 sat - inc EPO

6. What reveresible things can a sideroblastic anemia
Lysine for glutamate at position 6
Lead - and EtOH
Complications of sickle cell anemia
B6 therapy (pyrodixine)

7. What does bradykinin do?
Anti Rh
Inc vasodiltion - inc perm - inc pain
Factor V Leiden
Hereditary spherocytosis - autoimmune hemolysis

8. Drug that inhbits the GpIIb/IIIa directly
Varying shapes
Valine for glutamate
Antigen - antibody
Abciximab

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

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10. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Malaria - Babesia
Anti Rh
DIC - TTP/HUS - traumatic hemolysis
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

11. What is the pathogenesis of ACD?
Porphyrias
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Ringed sideronblasts with iron laden mitochondria
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

12. Bite cell
Prothrombin gene mutation
G6PD
Inc HbF and dec HbS
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

13. Serum iron - transferrin - ferritin lab values for iron def anemia
Down - up - down
Prothrombin gene mutation
Nl - up - nl
Howell - Jolly bodies - hypo/asplenia

14. RBCs damagaed from passing through obstructed - narrowed vessel lumina
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Thrombotic thrombocytopenic purpura
MAHA
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 -

15. What do the labs show for TTP?
Her next Rh+ fetus
ATIII def
Lysine for glutamate at position 6
Schistocytes and inc LDH

16. How are plasma cells characterized?
Helps platelts adhere to endothelium
Off center nuclues - clock face chromatin
Inc HbA2 on electrophoresis
GpIIb/IIIa

17. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
CLL (SLL without the peripheral lymphocytosis
Lung disease - congenital heart diseaes - and high altitude
Hydroxyurea - bone marrow transplant
Inc vasodiltion - inc perm - inc pain

18. What portion of IgE can mast cells bind
Ticlopidine/clopidogrel
Pyruvate kinase def - extravascular
Fc
T(9;22) bcr abl

19. Which drugs can cause macrocytic anemia?
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
No antigen - both antibodies
5- FU - AZT - hydroxyurea
B6 therapy (pyrodixine)

20. Acanthocyte (spur cell)
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Liver disease - abetalipoproteinemia - acntho = spiny
Deficiency in factor VIII
VWF carries/protects factor VIII

21. What is the treatment for sideroblastic anemia
HbSS
Fe def - thal - pb poison - sideroblastic anemia
B6 therapy (pyrodixine)
ADP and Ca

22. bcl -2 t(14;18) adults
No platelet clumping
Follicular lymphoma - indolent course
Inc large vWF multimers - inc platelet aggregation and thrombosis
Complications of sickle cell anemia

23. What is the clinical picture of hemophilia A or B
EBV
CML
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Macrohemorrhage - hemarthroses - easy bruising

24. Spherocyte
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Hereditary spherocytosis - autoimmune hemolysis
MHC II and Fc receptors
B cells

25. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Complications of sickle cell anemia
Bleeding - malnutrition/absorption - inc demand pregs
2 to 10
Heinz bodies - seen in alpha thal and G6PD

26. Who has more severe disease - HbSS or HbSC
HbSS
Sheets of lymphocytes interspersed with macs
Off center nuclues - clock face chromatin
2 to 10

27. How are the nucleus and the cytoplasm characterized for lymphocytes
Essential thrombocytosis
Round densly staining nucleus with a small amount of pale cytoplasm
ATIII def
Imatinib - anti bcr abl

28. What do you see a starry sky appearance in Burkitt
Dec
Sheets of lymphocytes interspersed with macs
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

29. What substance accumulates in porphyria cutanea
Uroporphyrin (tea colored urine)
Lysine for glutamate at position 6
Collagen - BM - activated platelets
Fibrinogen

30. poikilocytosis
Porphobilinogen - delta ALA - uroporphyrin
Varying shapes
Beta 4
Sickle cell anemia

31. What is the characteristic histologic finding in Hodgkin Lymphoma
Intrinsic - all factors except - VII - XIII
Reed - sternberg cells
Myelofibrosis (marow is crying being its fibrosed'
Imatinib - anti bcr abl

32. What CD molecules are on RS cells
HbSS
40 to 50
CML
CD15 and CD30 pos

33. Teardrop cell
Bone marrow infiltration - myelofibrosis
Idiopathic thrombocytopenia (ITP)
Lysosomes
CNS and testis

34. What is the pathogenesis of aplastic anemia with kidney disease
Malaria - Babesia
120 days
Decrease EPO
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

35. How does vWD cause elevated PTT?
Beta chain underproduced - asymptomatic - heterozygote
Sideroblastic anemia
VWF carries/protects factor VIII
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

36. What is contained within the azurophilic granules of PMNs
Nl - up - nl
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Fibrinogen bind GpIIb/IIIa and links platelts
Round densly staining nucleus with a small amount of pale cytoplasm

37. What is the affected enzyme in acute intermittment porphyria
Multiple myeloma
Porphobilinogen - delta ALA - uroporphyrin
Ewing sarcoma
Bimodal - men>women - except for nodular sclerosing type

38. What is the therapy for CML (philly chrom)
Intrinsic - all factors except - VII - XIII
B6 therapy (pyrodixine)
Imatinib - anti bcr abl
AML

39. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Easy gas exchange
Folate def

40. What virus is associated with 50% of Hodgkin lymphoma
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Dec
EBV
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

41. Fava beans - sufla drugs - infectinos
Extravascular
Valine for glutamate
Down - down - up
Examples of oxidative stress

42. What begins the extrinsic pathway?
Extravascular
Anemia of chronic disease - Aplastic anemia - kidney disease
Tissue factor converst the VII and VIIa
No lytic bone lesions in WM

43. What is the treatment for lead poising?
Varying sizes
Liver disease - abetalipoproteinemia - acntho = spiny
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Intrinsic - all factors except - VII - XIII

44. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
All
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Histaminase and arylsulfatase
Reed - sternberg cells

45. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Idiopathic thrombocytopenia (ITP)
CD15 and CD30 pos
Fibrinogen
Dec plasma volume

46. What are the neuro sx of B12 def?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Plasma cell
Round densly staining nucleus with a small amount of pale cytoplasm
Imatinib - anti bcr abl

47. What does CRAB stand for in multiple myeloma
A antigen and B antibodies
TPA - cleavage of fibrin mesh
Dec synthesis of factors 1972 - protein C/S
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

48. What are dendritic cells called in the skin?
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Langerhans cells
Valine for glutamate
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

49. What is the receptor for vWF
Gp1b
Intravascular hemolysis
B antigena and A antibodes
Plummer - vinson syndrome

50. What chromosomal translocation is associated with a better prognosis in All
T(12;21)
Incactivates II - VII - IX - X - XI - XII
Protoporphyrin (blood)
Signs and sx of aplastic anemia