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1. What is the coombs test results in AIHA?
Her next Rh+ fetus
Fe def - thal - pb poison - sideroblastic anemia
...
Pos

2. What is makes a leukemia acute
Folate def
Plummer - vinson syndrome
Blasts > 5%
Inc RBC - dec O2 sat - inc EPO

3. What is the treatment for acute intermittent porphyria
Deficiency in factor VIII
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Glucose and heme - which inhibit ALA synthase
Up - down - nl

4. What are the variants of the RS cells
Histamine - heparin - and eosinohil chemotactic factors
Lacunar in nodular sclerosing variant
Ticlopidine/clopidogrel
Inc UCB

5. Deficiency in GpIIb/IIIa

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6. Teardrop cell
Bone marrow infiltration - myelofibrosis
Megakaryocytes
Fibrinogen bind GpIIb/IIIa and links platelts
Tissue factor converst the VII and VIIa

7. Bone pain plus anemia
Sickle cell anemia
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Parvovirus
Multiple myeloma

8. What causes the physiologic chloride shift and What does the chloride shift do?
2 to 10
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Folate def
Positive osmootic fragility test and splenectomy

9. What finding you do you see in patients after splenectomy
Howell Jolly bodies
EBV
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Dec synthesis of factors 1972 - protein C/S

10. What is the age breakdown for hodgkins
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Bimodal - men>women - except for nodular sclerosing type
Reed - sternberg cells
Ferrocheletase and ALA dehydrogenase

11. What is the Ddx for for a macrocytic anemia
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Tissue factor converst the VII and VIIa
Dec
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

12. What is the pathogenesis of aplastic anemia with kidney disease
Decrease EPO
Low in CML
ADP and Ca
Polycythemia vera

13. deficiency in ADAMTS13 leading to dec degradation of vWF
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Hereditary spherocytosis - G6PD - sickle cell
Thrombotic thrombocytopenic purpura
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

14. What is the life spance of a platelet?
8-10 days
120 days
Inactivates it
40 to 50

15. universal recipient
Degrades fibrin mesh and converts C3 to C3a
Langerhans cells
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
AB - no antibodies

16. What is the most common nonhodgkin lymphoma
Lymphoma
Extrinsic - I - II - V - VII and X
Diffuse Large b cell lymphoma
MGUS - monoclonal gammopathy of undetermined significance

17. What does 'Neutrophils Like Making Everything Better' stand for?
M3 AML (acute promyelocytic leukemia)
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Lysine for glutamate at position 6
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

18. What is the activation stage of platelet plug formation?
T(12;21)
DDAVP (desmopressin) which releases stored vWF stored in endothelium
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Up - down - nl

19. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
Parvovirus
No platelet clumping
Hydroxyurea - bone marrow transplant

20. What converts plasminogen to plasm and What does plasmin do?
TPA - cleavage of fibrin mesh
Malaria - Babesia
B12/folate def
TRAP (tartrate resistant acid phosphatase

21. poikilocytosis
Low O2 in papilla; can also get microhematuria from medullary infarcts
Diffuse Large b cell lymphoma
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Varying shapes

22. What does NAACP stand for - in regards to the causes of eosinophiia?
Histaminase and arylsulfatase
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Produce antibody - lots of RER and golgi
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

23. t(11;14)
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
8-10 days
Mantle cell lymphoma
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

24. What do you see in peripheral smear in a pt with G6PD?
Megakaryocytes
Dec synthesis of factors 1972 - protein C/S
Bite cells and Heinz bodies
Inducers of primary antibody response

25. What is HbH
Paraprotein spike - monoclonal protein
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Beta 4
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

26. What is the receptor for vWF
AB - no antibodies
DIC
Hodgkin
Gp1b

27. What do the iron studies show in sideroblastic anemia
Von Willebrand's disease
Inc serum iron - normal TIBC - inc ferratin
...
Bone marrow - thymus - blood (80% of circulating lymphos are T)

28. What are the presenting symptoms of porphyria cutanea tarda
Myelofibrosis (marow is crying being its fibrosed'
Langerhans cells
Lung disease - congenital heart diseaes - and high altitude
Blistering cutaneous photosens - most common porphyria

29. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Factor V Leiden
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
B cells
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

30. What are some classic examples of extravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell
MAHA
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Bernard soulier

31. t(8;14) c - myc gene
Burkitt lymphoma
Africa = Jaw lesion - US = pelvis or abdomen
Dec plasma volume
Bleeding - malnutrition/absorption - inc demand pregs

32. What is hemophilia B?
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Def in factor IX
Off center nuclues - clock face chromatin
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

33. What is a blast crisis
CML to AML or All
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Howell Jolly bodies
AML

34. Why can newborns with sickle cell be asymptomatic
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Inc HbF and dec HbS

35. discrete tumor masses arising from lymph nodes
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Abciximab
Inc lymphoctes - and less RS cells
Lymphoma

36. How does the therapy for M3 vairant work?
Folate def
HbSS
Direct coombs - agglutinate if RBCs are coated with Ig
Induces differentiation of myeloblasts

37. In hemophilia A or B What do you see in the coag tests
Antigen - antibody
Nl PT - elevated PTT - intrinsic pathway defect
Rhogam - Rh antigen immunoglobulin
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

38. What is a metabolic disorder tht can cause macrocytic anemia?
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
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 -
Orotic aciduria
DIC

39. Blood type A
TRAP (tartrate resistant acid phosphatase
Ticlopidine/clopidogrel
Positive osmootic fragility test and splenectomy
A antigen and B antibodies

40. Schisotcyte - helmet cell
Ewing sarcoma
DIC - TTP/HUS - traumatic hemolysis
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Beta chain underproduced - asymptomatic - heterozygote

41. What are the presenting symptoms of acute intermittent porphyria
Extrinsic - I - II - V - VII and X
Hb Barts - gamma4 in defect in all 4 alpha genes
Birbeck granules
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

42. Target cell

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43. What activates the fibrinolytic pathway?
Lysine for glutamate at position 6
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Folate def
Blistering cutaneous photosens - most common porphyria

44. Drug that inhibits COX and therefore TXA2 synthesis
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Aspirin
All

45. What symptoms are associated with Hodgkin lymphoma
B symptoms - fever night sweats - weight loss
2 to 10
Low in CML
VIII

46. What is the difference of presentation of Burkitt in Africa vs the United States
Lysosomes
Africa = Jaw lesion - US = pelvis or abdomen
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

47. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Epoxide reductase - warfarin inhibits
G6PD
Off center nuclues - clock face chromatin
B symptoms - fever night sweats - weight loss

48. Deficiency in GpIb
B6
Birbeck granules
Beta 4
Bernard soulier

49. Which immunoglobulin is involved in warm agglutination?
Multiple myeloma
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
IgG - Warm is GREAT
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

50. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
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 -
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Yes - via MHC II
Paroxysmal nocturnal hemoglobinuria

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