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Answer 50 questions in 15 minutes.
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1. What are the four levels of alpha thal?
A antigen and B antibodies
Splicing sites and promotor sequences
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Inc HbA2 on electrophoresis

2. What is the therapy for CML (philly chrom)
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Bernard soulier
Imatinib - anti bcr abl
Cromolyn sodium

3. What is the are the presenting symptoms of lead poisoning in kids and adults
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
CNS and testis
Mycosis fundgoides/Sezary syndrome
Schistocytes - helmet cells

4. What is the pathogenesis of TTP?
Eleveated PT - PTT
Lymphoma
G6PD
Inc large vWF multimers - inc platelet aggregation and thrombosis

5. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Intravascular hemolysis
120 days
Histaminase and arylsulfatase
Round densly staining nucleus with a small amount of pale cytoplasm

6. universal donor
Mantle cell - older males
No
Fibrinogen bind GpIIb/IIIa and links platelts
No antigen - both antibodies

7. What is the characteristic histologic finding in Hodgkin Lymphoma
Hb Barts - gamma4 in defect in all 4 alpha genes
All trans retinoic acid
Reed - sternberg cells
Factor V resistant to activated protein C's inhibition

8. defect in alpha globin gene - dec alpha globin synth - disease and populations?
TRAP (tartrate resistant acid phosphatase
Beta chain underproduced - asymptomatic - heterozygote
HbSS
Alpha thal - asian and african american

9. What is the treatment for lead poising?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Mantle cell lymphoma
HbSS
M3 AML (acute promyelocytic leukemia)

10. anti - Ig antibody added to patients RBCs;
Alpha thal - asian and african american
Direct coombs - agglutinate if RBCs are coated with Ig
Imatinib - anti bcr abl
Decrease EPO

11. Eosinophils are highly phagocytic For what kind of complex?
Antigen - antibody
AB - no antibodies
Contiguous
Collagen - BM - activated platelets

12. Causes of iron def?
Leukemia
Bleeding - malnutrition/absorption - inc demand pregs
AML
Down - down - up

13. Basophilic nuclear remnants fonud in RBCs
Antigen - antibody
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Hodgkin
Howell - Jolly bodies - hypo/asplenia

14. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Inc HbA2 on electrophoresis
Intravascular hemolysis
Eleveated PT - PTT
Bimodal - men>women - except for nodular sclerosing type

15. What do eosinophils defend against and What do they use to do it?
Anemia of chronic disease - Aplastic anemia - kidney disease
Nodular sclerosing
Antigen - antibody
Helminth infections major basic protein

16. What activates the fibrinolytic pathway?
Paroxysmal nocturnal hemoglobinuria
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Lung disease - congenital heart diseaes - and high altitude
Porphobilinogen deaminase aka uroporphyrinogen I synthase

17. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
B12 def
Plummer - vinson syndrome
Blistering cutaneous photosens - most common porphyria
Lysine for glutamate at position 6

18. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Anti Rh
Acts to stabilize platelet plug
Bite cells and Heinz bodies
Dendritic cells?

19. What is the philadelphia chromosome
A antigen and B antibodies
Up - down - nl
Howell Jolly bodies
T(9;22) bcr abl

20. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
VIII
Helps platelts adhere to endothelium
Low in CML
Inducers of primary antibody response

21. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Sideroblastic anemia
No
CLL (SLL without the peripheral lymphocytosis
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

22. Plasma cell neoplasm
Dendritic cells?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
DIC - TTP/HUS - traumatic hemolysis
Multiple myeloma

23. Which factor does vWF carry/protect
VIII
Factor V resistant to activated protein C's inhibition
Degrades fibrin mesh and converts C3 to C3a
8-10 days

24. Which pathway and factors are tested in the PTT test
Anemia of chronic disease - Aplastic anemia - kidney disease
Both alpha and beta thal
Intrinsic - all factors except - VII - XIII
GpIIb/IIIa

25. What is the activation stage of platelet plug formation?
Myelofibrosis (marow is crying being its fibrosed'
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
GpIIb/IIIa

26. What is the enzyme defect in Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
VWF carries/protects factor VIII
Inactivates it
Splicing sites and promotor sequences

27. What causes the jaundice in extravascular hemolysis
Inc UCB
Beta chain underproduced - asymptomatic - heterozygote
B12 in fatty acid pathways leads to subacute combined degeneration
Cromolyn sodium

28. What is the general pathology of a macrocytic anemia?
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
They bind vWF via GpIb
Malaria - Babesia
Inc large vWF multimers - inc platelet aggregation and thrombosis

29. What is the ddx for aplastic anemia

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30. Back pain - hemoglobinuria
B12 in fatty acid pathways leads to subacute combined degeneration
GpIIb/IIIa
Lead - and EtOH
Common a few days after oxidative stress in a pt with G6PD

31. Elliptocyte
Mycosis fundgoides/Sezary syndrome
Hereditary elloptocytosis
Intravascular
Schistocytes - helmet cells

32. Serum iron - transferrin - ferritin lab values for hemochromatosis
Multiple myeloma
Factor V Leiden
Up - down - up
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 -

33. What are the variants of the RS cells
Lacunar in nodular sclerosing variant
Bleeding - malnutrition/absorption - inc demand pregs
Decrease EPO
DIC

34. Ringed sideroblasts
Sideroblastic anemia
CML to AML or All
Extrinsic - I - II - V - VII and X
AB - no antibodies

35. What is their role?
Def in factor IX
Inducers of primary antibody response
B6
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 -

36. Who are the professional APCs?
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Dendritic cells?
Extravascular hemolysis
M3 AML (acute promyelocytic leukemia)

37. What is the effect of ACE on bradykinin
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Extravascular
Inactivates it
Beta chain underproduced - asymptomatic - heterozygote

38. What are the azuraphilic granules in PMNs
B symptoms - fever night sweats - weight loss
Parvovirus
Alpha thal - asian and african american
Lysosomes

39. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Incactivates II - VII - IX - X - XI - XII
G6PD
DIC
HIV or immunosupression

40. What percentage if WBCs are polys?
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Sideroblastic anemia
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
40 to 75

41. What is the receptor for vWF
MAHA
Helps platelts adhere to endothelium
20 to 40
Gp1b

42. What reveresible things can a sideroblastic anemia
Abciximab
Lead - and EtOH
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
120 days

43. What is the difference of presentation of Burkitt in Africa vs the United States
Blistering cutaneous photosens - most common porphyria
Plasma cell
Paroxysmal nocturnal hemoglobinuria
Africa = Jaw lesion - US = pelvis or abdomen

44. What is the accumulated substance in lead poisoning
Protoporphyrin (blood)
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Signs and sx of aplastic anemia
Splicing sites and promotor sequences

45. Where do you see hypersegmented polys?
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
B12/folate def
Down - up - down
Thrombotic thrombocytopenic purpura

46. What does the large SA:volume ratio in RBCs help facilitate?
Folate def
Ankryin - band 4.1 or spectrin
Leukemia
Easy gas exchange

47. What do the platelets bind? What is the step called
Mantle cell - older males
Contiguous
They bind vWF via GpIb
Gp1b

48. What does increasing heme do to ALA synthase activity
Orotic aciduria
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Dec

49. What CD molecules are on RS cells
CNS and testis
CD15 and CD30 pos
Lysine for glutamate at position 6
Hodgkin

50. S-100 and CD1a with birbeck granules
Hereditary elloptocytosis
No platelet clumping
Protein C or S deficiency
Langerhans cell histiocytosis

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