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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 HbH
Sheets of lymphocytes interspersed with macs
Anemia of chronic disease - Aplastic anemia - kidney disease
Beta 4
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

2. Serum iron - transferrin - ferritin lab values for pregs - OCP use
G6PD
Schistocytes - helmet cells
Nl - up - nl
Aplastic anemia - pancytopenia

3. What is the defect in beta thal?
Sideroblastic anemia
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Von Willebrand's disease
Splicing sites and promotor sequences

4. What do you see in vit K def
Inducers of primary antibody response
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
2 to 10
Eleveated PT - PTT

5. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Beta thal
Histamine - heparin - and eosinohil chemotactic factors
No antigen - both antibodies
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

6. What do the dense granules contain in platelets
ADP and Ca
Lead - and EtOH
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Inc vasodiltion - inc perm - inc pain

7. What are the presenting symptoms of acute intermittent porphyria
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Nl PT - elevated PTT - intrinsic pathway defect
Blasts > 5%
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

8. What reveresible things can a sideroblastic anemia
MAHA
Mantle cell - older males
20 to 40
Lead - and EtOH

9. What signal activates MACS
IFN gama
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Inc HbA2 on electrophoresis

10. What is the tx for aplastic anemia
Hb Barts - gamma4 in defect in all 4 alpha genes
Sideroblastic anemia
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Schistocytes and inc LDH

11. What does 'Neutrophils Like Making Everything Better' stand for?
Sideroblastic anemia
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
40 to 75
Low in CML

12. What is the affected enzyme in acute intermittment porphyria
Imatinib - anti bcr abl
Porphobilinogen - delta ALA - uroporphyrin
No
Hodgkin

13. What condition can result from treating AML M3 from the release of the Auer rods
DIC
CD15 and CD30 pos
All
Mantle cell - older males

14. Teardrop cell
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
IFN gama
Bone marrow infiltration - myelofibrosis
Hereditary spherocytosis - G6PD - sickle cell

15. What do they express on their surface?
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Signs and sx of aplastic anemia
DIC - TTP/HUS - traumatic hemolysis
MHC II and Fc receptors

16. What is the age group most commonly affected by multiple myeloma
Pyruvate kinase def - extravascular
Beta chain underproduced - asymptomatic - heterozygote
Dendritic cells?
40 to 50

17. What is the effected enzyme in acute intermittment porphyria?
Hb Barts - gamma4 in defect in all 4 alpha genes
Aspirin
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Schistocytes and inc LDH

18. Schisotcyte - helmet cell
DIC - TTP/HUS - traumatic hemolysis
Inc UCB
CML
IgG - Warm is GREAT

19. What happens in betal thal minor?
Beta chain underproduced - asymptomatic - heterozygote
No platelet clumping
Langerhans cells
40 to 50

20. What does Vit K deficiency cause?
Dec synthesis of factors 1972 - protein C/S
A antigen and B antibodies
Nl PT - elevated PTT - intrinsic pathway defect
Hydroxyurea - bone marrow transplant

21. t(15;17)
VWF carries/protects factor VIII
MGUS - monoclonal gammopathy of undetermined significance
Africa = Jaw lesion - US = pelvis or abdomen
M3 AML (acute promyelocytic leukemia)

22. What is the characteristic finding for MAHA on peripheral smear?
Myelofibrosis (marow is crying being its fibrosed'
Up - down - nl
T(12;21)
Schistocytes - helmet cells

23. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Rhogam - Rh antigen immunoglobulin
Reed - sternberg cells
Alpha thal - asian and african american

24. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Her next Rh+ fetus
Exposed collagen upon endothelial damage
Ankryin - band 4.1 or spectrin

25. Deficiency in GpIIb/IIIa

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26. What do labs show in DIC?
T(9;22) bcr abl
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Multiple myeloma
Anemia of chronic disease - Aplastic anemia - kidney disease

27. Mutation in 3' untranslated region associated with venous clots
Prothrombin gene mutation
Pyruvate kinase def - extravascular
Mantle cell lymphoma
Def in factor IX

28. What does ectopic EPO produce
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
PGI2 - NO inc blood flow - dec platelet aggregation
Inappropriate absolute with inc RBCs and EPO

29. What does antithrombin do and What activates it?
Inc serum iron - normal TIBC - inc ferratin
Spleen
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Macrohemorrhage - hemarthroses - easy bruising

30. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Hereditary spherocytosis
Up - down - nl
Von Willebrand's disease
EBV

31. What do you see in peripheral smear in a pt with G6PD?
Low O2 in papilla; can also get microhematuria from medullary infarcts
Bite cells and Heinz bodies
No antigen - both antibodies
A antigen and B antibodies

32. What is the mutation in HbC
B12/folate def
Intrinsic - all factors except - VII - XIII
Degrades fibrin mesh and converts C3 to C3a
Lysine for glutamate at position 6

33. anisocytosis
Def in factor IX
Varying sizes
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
A antigen and B antibodies

34. Which drugs can cause macrocytic anemia?
Yes - via MHC II
G6PD
5- FU - AZT - hydroxyurea
Tissue factor converst the VII and VIIa

35. What is the most common hodgkin lymphoma
CNS and testis
90% anearobically from glucose to lactate - 10% from HMP shunt
Imatinib - anti bcr abl
Nodular sclerosing

36. What is the effect of ACE on bradykinin
Inc lymphoctes - and less RS cells
Inactivates it
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Lung disease - congenital heart diseaes - and high altitude

37. What causes the jaundice in extravascular hemolysis
Inc UCB
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Leukemia
Protoporphyrin

38. Basophilica stippling
Langerhans cells
Sheets of lymphocytes interspersed with macs
Direct coombs - agglutinate if RBCs are coated with Ig
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

39. Which substrates begin the heme synthesis pathway
Exposed collagen upon endothelial damage
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 -
Nl PT - elevated PTT - intrinsic pathway defect
Glycine and succinyl - coa

40. Where are 1/3 of platelets stored
Anemia of chronic disease - Aplastic anemia - kidney disease
Anti Rh
Protein S - cleaves and invactivates Va and VIIIa
Spleen

41. Elderly - mature b cell tumor with filamentous - hairlike projections
Nl - up - nl
Paraprotein spike - monoclonal protein
Hairy cell leukemia
B symptoms - fever night sweats - weight loss

42. Blood type A
No
A antigen and B antibodies
Beta thal
ATIII def

43. X linked - dec glutathione inc RBC susceptibility to oxidative stress
G6PD
PGI2 - NO inc blood flow - dec platelet aggregation
AB - no antibodies
Plummer - vinson syndrome

44. What is the life span of a normal RBC
Factor V Leiden
120 days
They bind vWF via GpIb
Leukemoid reaction

45. Deficiency in GpIb
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 -
Ferrocheletase and ALA dehydrogenase
Bernard soulier
Low O2 in papilla; can also get microhematuria from medullary infarcts

46. Fava beans - sufla drugs - infectinos
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Examples of oxidative stress
M3 AML (acute promyelocytic leukemia)
Alpha thal - asian and african american

47. What does bradykinin do?
Parvovirus
ADP and Ca
Inc vasodiltion - inc perm - inc pain
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 -

48. How does the therapy for M3 vairant work?
Petechiae
Fibrinogen
Orotic aciduria
Induces differentiation of myeloblasts

49. What is the difference of presentation of Burkitt in Africa vs the United States
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Africa = Jaw lesion - US = pelvis or abdomen
Extravascular
Inc vasodiltion - inc perm - inc pain

50. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
VWF and fibrinogen
Africa = Jaw lesion - US = pelvis or abdomen
Idiopathic thrombocytopenia (ITP)
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis