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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 platelets interact with to form a hemostatic plug
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Mantle cell lymphoma
Fibrinogen
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

2. How is beta thal minor dx
Inc HbA2 on electrophoresis
All
HIV or immunosupression
Abciximab

3. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Sideroblastic anemia
Examples of oxidative stress
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Porphyrias

4. What causes hydrops fetalis
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Acts to stabilize platelet plug
Hb Barts - gamma4 in defect in all 4 alpha genes
Leukemia

5. universal recipient
Tissue factor converst the VII and VIIa
Up - down - nl
AB - no antibodies
Extravascular hemolysis

6. What is the receptor for fibrinogen?
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Porphobilinogen - delta ALA - uroporphyrin
Nl PT - elevated PTT - intrinsic pathway defect
GpIIb/IIIa

7. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Inc HbF and dec HbS
CLL (SLL without the peripheral lymphocytosis
DIC - TTP/HUS - traumatic hemolysis
VIII

8. What happens in betal thal minor?
Beta chain underproduced - asymptomatic - heterozygote
Birbeck granules
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
They bind vWF via GpIb

9. What is a metabolic disorder tht can cause macrocytic anemia?
Intravascular
A antigen and B antibodies
B6
Orotic aciduria

10. Where do B cells arise from - mature - and migrate to...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Inc RBC - dec O2 sat - inc EPO
T(12;21)

11. What is the treatment for acute intermittent porphyria
Imatinib - anti bcr abl
Common a few days after oxidative stress in a pt with G6PD
Glucose and heme - which inhibit ALA synthase
Inc HbA2 on electrophoresis

12. What role does antithrombin play?
B antigena and A antibodes
TPA - cleavage of fibrin mesh
Incactivates II - VII - IX - X - XI - XII
Hodgkin

13. What is the treatment for sideroblastic anemia
Schistocytes - helmet cells
B6 therapy (pyrodixine)
Complications of sickle cell anemia
Glanzmann's throbmasthenia

14. Why does B12 def cause neuro sx?
B12 in fatty acid pathways leads to subacute combined degeneration
1 to 6
Acts to stabilize platelet plug
VWF carries/protects factor VIII

15. Who are the professional APCs?
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
20 to 40
Direct coombs - agglutinate if RBCs are coated with Ig
Dendritic cells?

16. What are some causes of warm agglutinin autoimmune hemolytic anemia?
HIV or immunosupression
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Pos
SLE - CLL - alpha methyldopa

17. What activates the fibrinolytic pathway?
Protein C or S deficiency
Bone marrow infiltration - myelofibrosis
Abciximab
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

18. What is their role?
Inducers of primary antibody response
Africa = Jaw lesion - US = pelvis or abdomen
Extrinsic - I - II - V - VII and X
ATIII def

19. Serum iron - transferrin - ferritin lab values for iron def anemia
Gp1b
Down - up - down
Aspirin
DIC

20. Back pain - hemoglobinuria
IgG - Warm is GREAT
Common a few days after oxidative stress in a pt with G6PD
Acts to stabilize platelet plug
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

21. What are some classic examples of extravascular hemolysis
Gp1b
Orotic aciduria
Hereditary spherocytosis - G6PD - sickle cell
M3 AML (acute promyelocytic leukemia)

22. What are the presenting symptoms of acute intermittent porphyria
Easy gas exchange
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
No antigen - both antibodies
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

23. hemolytic in a newborn - dec ATP and rigid RBCs
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Down - down - up
Tissue factor converst the VII and VIIa
Pyruvate kinase def - extravascular

24. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Intravascular hemolysis
Prothrombin gene mutation
Varying shapes
Low in CML

25. Fava beans - sufla drugs - infectinos
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Hairy cell leukemia
Examples of oxidative stress
Blistering cutaneous photosens - most common porphyria

26. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Paroxysmal nocturnal hemoglobinuria
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Africa = Jaw lesion - US = pelvis or abdomen
Gp1b

27. What virus can cause an aplastic crisis in pts with HS?
Parvovirus
Inc HbF and dec HbS
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Tissue

28. What do you see in peripheral smear in a pt with G6PD?
B6
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Bite cells and Heinz bodies
Alpha thal - asian and african american

29. poikilocytosis
Varying shapes
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Inactivates it
T(9;22) bcr abl

30. What are the presenting symptoms of porphyria cutanea tarda
Hydroxyurea - bone marrow transplant
Blistering cutaneous photosens - most common porphyria
Folate def
Degrades fibrin mesh and converts C3 to C3a

31. X linked - dec glutathione inc RBC susceptibility to oxidative stress
G6PD
Blood
VIII
40 to 75

32. Elderly - mature b cell tumor with filamentous - hairlike projections
Splicing sites and promotor sequences
Ferrocheletase and ALA dehydrogenase
Hairy cell leukemia
Inc

33. What is the ddx for aplastic anemia

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34. What is hemophilia B?
Pyruvate kinase def - extravascular
Def in factor IX
Inc lymphoctes - and less RS cells
Peroxidase

35. Which maternal antibodies cross the placenta - anti A/B or anti Rh
T(12;21)
Degrades fibrin mesh and converts C3 to C3a
Plummer - vinson syndrome
Anti Rh

36. What is the are the presenting symptoms of lead poisoning in kids and adults
T(12;21)
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Pos

37. What do the platelets bind? What is the step called
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Aspirin
B antigena and A antibodes
They bind vWF via GpIb

38. After an injury - What does vWF bind to begin platelet plug formation?
No platelet clumping
Exposed collagen upon endothelial damage
Peroxidase
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

39. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Up - down - nl
Essential thrombocytosis
Malaria - Babesia
IgG - Warm is GREAT

40. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Prothrombin gene mutation
120 days
Polycythemia vera
20 to 40

41. What is the main source of energy in RBCs
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
DIC - TTP/HUS - traumatic hemolysis
90% anearobically from glucose to lactate - 10% from HMP shunt
Fibrinogen bind GpIIb/IIIa and links platelts

42. discrete tumor masses arising from lymph nodes
Lymphoma
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
2 to 10
Fc

43. What are the likely exposures of kids and adults for lead poisoning
Kids= exposure to lead paint - adults = battery - ammunition factory
Blistering cutaneous photosens - most common porphyria
Glanzmann's throbmasthenia
Langerhans cells

44. What is the accumulated substance in lead poisoning
Contiguous
120 days
Protoporphyrin (blood)
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

45. What do labs show in DIC?
Intravascular hemolysis
B6
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

46. What are the labs and tx for HS?
Histamine - heparin - and eosinohil chemotactic factors
EBV
Positive osmootic fragility test and splenectomy
Kids= exposure to lead paint - adults = battery - ammunition factory

47. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Nl - up - nl
Sickle cell
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Parvovirus

48. What indicated a good prognosis in Hodgkin lymphoma?
Protoporphyrin
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Anti Rh
Inc lymphoctes - and less RS cells

49. What is the mutation in HbC
Lysine for glutamate at position 6
1 to 6
CML
Inc RBC - dec O2 sat - inc EPO

50. What symptoms are associated with Hodgkin lymphoma
B symptoms - fever night sweats - weight loss
SLE - CLL - alpha methyldopa
Lacunar in nodular sclerosing variant
Factor V Leiden