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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. Mutation in 3' untranslated region associated with venous clots
Prothrombin gene mutation
Porphobilinogen - delta ALA - uroporphyrin
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Blod - tissue - MACS

2. In hemophilia A or B What do you see in the coag tests
Aspirin
Nl PT - elevated PTT - intrinsic pathway defect
Cromolyn sodium
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

3. iron deficiency anemia - esophageal web - atrophic glossitis
Uroporphyrinogen decarboxylase
Ferrocheletase and ALA dehydrogenase
Plummer - vinson syndrome
Dec

4. What are the presenting symptoms of acute intermittent porphyria
Schistocytes and inc LDH
Low in CML
Incactivates II - VII - IX - X - XI - XII
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

5. What are the etiologies of B12 def
EBV
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
PGI2 - NO inc blood flow - dec platelet aggregation
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

6. What is the ddx for aplastic anemia

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7. What vitamin is a cofactor for the first step of heme synthesis
Helminth infections major basic protein
Hereditary spherocytosis
B6
Hb Barts - gamma4 in defect in all 4 alpha genes

8. hypocellular bone marrown tih fatty infiltration
Aplastic anemia - pancytopenia
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Ticlopidine/clopidogrel
Inappropriate absolute with inc RBCs and EPO

9. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Signs and sx of aplastic anemia
Helps platelts adhere to endothelium
Her next Rh+ fetus

10. What is factor V leidin?

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11. What do you see in vit K def
ADP and Ca
Inc
Porphobilinogen - delta ALA - uroporphyrin
Eleveated PT - PTT

12. What does the large SA:volume ratio in RBCs help facilitate?
Sideroblastic anemia
Complications of sickle cell anemia
Easy gas exchange
Mycosis fundgoides/Sezary syndrome

13. Wilm's tumor - RCC - HCC - hydronephrosis
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Yes - via MHC II
40 to 75

14. HTLV-1
CLL (SLL without the peripheral lymphocytosis
Sickle cell anemia
Folate def
Adult T cell lymphoma - presents with cutaneous lesions

15. What does plasmin do?
GpIIb/IIIa
Sideroblastic anemia
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Degrades fibrin mesh and converts C3 to C3a

16. mother's antibodies attack fetal RBCs
Schistocytes and inc LDH
Intrinsic - all factors except - VII - XIII
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

17. Back pain - hemoglobinuria
No antigen - both antibodies
Common a few days after oxidative stress in a pt with G6PD
40 to 75
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

18. What are the likely exposures of kids and adults for lead poisoning
Dec
Epoxide reductase - warfarin inhibits
Kids= exposure to lead paint - adults = battery - ammunition factory
B12 in fatty acid pathways leads to subacute combined degeneration

19. Why does B12 def cause neuro sx?
Sheets of lymphocytes interspersed with macs
B12 in fatty acid pathways leads to subacute combined degeneration
Deficiency in factor VIII
No

20. t(8;14) c - myc gene
HIV or immunosupression
Hereditary spherocytosis - G6PD - sickle cell
Burkitt lymphoma
TPA - cleavage of fibrin mesh

21. universal donor
Inc lymphoctes - and less RS cells
No antigen - both antibodies
B symptoms - fever night sweats - weight loss
Beta chain underproduced - asymptomatic - heterozygote

22. What is the tx for aplastic anemia
Def in factor IX
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Lysosomes
Lysine for glutamate at position 6

23. Sickle cell
Sickle cell anemia
Fc
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Produce antibody - lots of RER and golgi

24. What do plasma cells do
Pos
Hereditary spherocytosis - G6PD - sickle cell
Produce antibody - lots of RER and golgi
Sideroblastic anemia

25. When do you see MAHA?
Lysine for glutamate at position 6
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Africa = Jaw lesion - US = pelvis or abdomen

26. What is the treatment for lead poising?
120 days
Extravascular hemolysis
Thrombotic thrombocytopenic purpura
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

27. What are the labs and tx for HS?
Decrease EPO
Positive osmootic fragility test and splenectomy
Extrinsic - I - II - V - VII and X
All

28. What is hemophiliia A
Deficiency in factor VIII
Dec
Howell - Jolly bodies - hypo/asplenia
Splicing sites and promotor sequences

29. deficiency in ADAMTS13 leading to dec degradation of vWF
Mantle cell lymphoma
Protein C or S deficiency
Hereditary elloptocytosis
Thrombotic thrombocytopenic purpura

30. normal RBCs added to patient's serum
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Lead - and EtOH
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Indirect coombs - agglutinate if serum anti RBC surface Ig

31. What is the mutation in HbS
Valine for glutamate
MAHA
Blasts > 5%
No

32. Where are mast cells found
Easy gas exchange
Tissue
120 days
Malaria - Babesia

33. What happens in beta thal major?
Intrinsic - all factors except - VII - XIII
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Ringed sideronblasts with iron laden mitochondria
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

34. What are dendritic cells called in the skin?
EBV
Porphyrias
B12/folate def
Langerhans cells

35. What is the clinical picture of hemophilia A or B
Multiple myeloma
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Macrohemorrhage - hemarthroses - easy bruising
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

36. hemolytic in a newborn - dec ATP and rigid RBCs
IFN gama
Lacunar in nodular sclerosing variant
Dendritic cells?
Pyruvate kinase def - extravascular

37. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
HIV or immunosupression
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
B6
T(9;22) bcr abl

38. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Nl PT - elevated PTT - intrinsic pathway defect
Inc vasodiltion - inc perm - inc pain
Porphyrias

39. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
Sickle cell
TRAP (tartrate resistant acid phosphatase
Extravascular
Prothrombin gene mutation

40. t(11:22)
Rhogam - Rh antigen immunoglobulin
Ewing sarcoma
B12 in fatty acid pathways leads to subacute combined degeneration
AML

41. What do you see a starry sky appearance in Burkitt
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Von Willebrand's disease
Sheets of lymphocytes interspersed with macs
40 to 75

42. What is the tx for vWD
Beta thal
Porphobilinogen deaminase aka uroporphyrinogen I synthase
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Dendritic cells?

43. What do the iron studies show in sideroblastic anemia
Inc serum iron - normal TIBC - inc ferratin
120 days
Varying shapes
Schistocytes - helmet cells

44. What are the four levels of alpha thal?
Up - down - nl
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
MAHA
Ticlopidine/clopidogrel

45. What do they express on their surface?
Plasma cell
VWF and fibrinogen
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
MHC II and Fc receptors

46. What is the Ddx for a normocytic - normochromic anemia?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Polycythemia vera
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Uroporphyrinogen decarboxylase

47. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Petechiae
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Factor V Leiden
Beta chain underproduced - asymptomatic - heterozygote

48. Serum iron - transferrin - ferritin lab values for iron def anemia
Anti Rh
Down - up - down
Factor V resistant to activated protein C's inhibition
Nl - up - nl

49. What do platelets interact with to form a hemostatic plug
Alpha thal - asian and african american
Fibrinogen
Hereditary spherocytosis - G6PD - sickle cell
Extravascular

50. What do the labs show for TTP?
Mycosis fundgoides/Sezary syndrome
CNS and testis
Schistocytes and inc LDH
Glycine and succinyl - coa