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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. Where are 1/3 of platelets stored
Leukemoid reaction
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Bernard soulier
Spleen

2. Spherocyte
Up - down - nl
Hereditary spherocytosis - autoimmune hemolysis
Deficiency in factor VIII
Protein S - cleaves and invactivates Va and VIIIa

3. What signal activates MACS
IFN gama
Aplastic anemia - pancytopenia
Hairy cell leukemia
CML to AML or All

4. What is the activation stage of platelet plug formation?
Intravascular
DIC
Splicing sites and promotor sequences
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

5. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Idiopathic thrombocytopenia (ITP)
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Essential thrombocytosis
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

6. drug that inhibits ADP induced expression of GpIIb/IIIa
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Ticlopidine/clopidogrel
Plasma cell
Tissue factor converst the VII and VIIa

7. What does CRAB stand for in multiple myeloma
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
PGI2 - NO inc blood flow - dec platelet aggregation
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

8. What do you see on peripheral smear with sideroblastic anemia
They bind vWF via GpIb
Ringed sideronblasts with iron laden mitochondria
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
DDAVP (desmopressin) which releases stored vWF stored in endothelium

9. What are the etiologies of folate def
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
8-10 days
Direct coombs - agglutinate if RBCs are coated with Ig
Intrinsic - all factors except - VII - XIII

10. What is the presenting scenario for TTP?
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Howell Jolly bodies
ADP and Ca
Alpha thal - asian and african american

11. inc HbF on electrophoresis
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Up - down - up
MAHA
Both alpha and beta thal

12. philadelphia chromosome - blood looks like marrow
1 to 6
CML
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
MGUS - monoclonal gammopathy of undetermined significance

13. hypocellular bone marrown tih fatty infiltration
Aplastic anemia - pancytopenia
Collagen - BM - activated platelets
Histaminase and arylsulfatase
Varying shapes

14. When is the peak incidence for nonHod lymphoma
B12/folate def
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Low in CML
20 to 40

15. What is the enzyme defect in Porphyria cutanea tarda
Inc vasodiltion - inc perm - inc pain
Uroporphyrinogen decarboxylase
Protoporphyrin (blood)
DIC

16. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Porphyrias
Leukemoid reaction
Paroxysmal nocturnal hemoglobinuria
Mantle cell lymphoma

17. What is the mutation in HbS
VWF carries/protects factor VIII
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Abciximab
Valine for glutamate

18. What is hemophilia B?
Liver disease - abetalipoproteinemia - acntho = spiny
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
No lytic bone lesions in WM
Def in factor IX

19. What are the age ranges for the various leukemias
Inducers of primary antibody response
Ticlopidine/clopidogrel
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Up - down - up

20. Where are basophils found?
Uroporphyrinogen decarboxylase
Hb Barts - gamma4 in defect in all 4 alpha genes
EBV
Blood

21. Which infections can cause MAHA?
Both alpha and beta thal
IFN gama
Malaria - Babesia
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

22. How are the nucleus and the cytoplasm characterized for lymphocytes
Round densly staining nucleus with a small amount of pale cytoplasm
VIII
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Contiguous

23. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Paraprotein spike - monoclonal protein
DIC
Low in CML
Common a few days after oxidative stress in a pt with G6PD

24. After an injury - What does vWF bind to begin platelet plug formation?
A antigen and B antibodies
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Exposed collagen upon endothelial damage
Helminth infections major basic protein

25. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Hb Barts - gamma4 in defect in all 4 alpha genes
Her next Rh+ fetus

26. What virus is associated with 50% of Hodgkin lymphoma
Inc lymphoctes - and less RS cells
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Epoxide reductase - warfarin inhibits
EBV

27. What are the etiologies of B12 def
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Anti Rh
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Paroxysmal nocturnal hemoglobinuria

28. What the alpha granules contain in platelets?
SLE - CLL - alpha methyldopa
VWF and fibrinogen
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Helminth infections major basic protein

29. What condition can result from treating AML M3 from the release of the Auer rods
DIC
Varying shapes
Paroxysmal nocturnal hemoglobinuria
Kids= exposure to lead paint - adults = battery - ammunition factory

30. What substances are released in mast cell degranulation
Yes - via MHC II
Paroxysmal nocturnal hemoglobinuria
CML to AML or All
Histamine - heparin - and eosinohil chemotactic factors

31. What causes hydrops fetalis
Hb Barts - gamma4 in defect in all 4 alpha genes
Aplastic anemia - pancytopenia
Sideroblastic anemia
DIC - TTP/HUS - traumatic hemolysis

32. CD5+ - poor prognosis - t(11;14)
No platelet clumping
Mantle cell - older males
CLL (SLL without the peripheral lymphocytosis
Up - down - up

33. What portion of IgE can mast cells bind
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Fc
Abciximab
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

34. X linked - dec glutathione inc RBC susceptibility to oxidative stress
AB - no antibodies
Low O2 in papilla; can also get microhematuria from medullary infarcts
Examples of oxidative stress
G6PD

35. deficiency in ADAMTS13 leading to dec degradation of vWF
Thrombotic thrombocytopenic purpura
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Histaminase and arylsulfatase
Birbeck granules

36. What role does antithrombin play?
Incactivates II - VII - IX - X - XI - XII
Antigen - antibody
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

37. What finding you do you see in patients after splenectomy
Spleen
Antigen - antibody
Howell Jolly bodies
Abciximab

38. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
Bleeding - malnutrition/absorption - inc demand pregs
Kids= exposure to lead paint - adults = battery - ammunition factory
B6

39. What reveresible things can a sideroblastic anemia
Sheets of lymphocytes interspersed with macs
Hereditary elloptocytosis
Gp1b
Lead - and EtOH

40. Drug that inhbits the GpIIb/IIIa directly
Complications of sickle cell anemia
Inc vasodiltion - inc perm - inc pain
Blasts > 5%
Abciximab

41. What is the age breakdown for hodgkins
Extravascular
Positive osmootic fragility test and splenectomy
Valine for glutamate
Bimodal - men>women - except for nodular sclerosing type

42. What is the general pathology of a macrocytic anemia?
Diffuse Large b cell lymphoma
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

43. inc LDH - jaundice
Macrohemorrhage - hemarthroses - easy bruising
Extravascular hemolysis
Factor V Leiden
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

44. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Up - down - nl
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Uroporphyrinogen decarboxylase
MHC II and Fc receptors

45. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
Her next Rh+ fetus
Dec
Sideroblastic anemia
No lytic bone lesions in WM

46. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Hairy cell leukemia
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Signs and sx of aplastic anemia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

47. Basophilic nuclear remnants fonud in RBCs
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Howell - Jolly bodies - hypo/asplenia
Spleen
2 to 10

48. Fava beans - sufla drugs - infectinos
TRAP (tartrate resistant acid phosphatase
Factor V Leiden
Examples of oxidative stress
CLL (SLL without the peripheral lymphocytosis

49. RBCs damagaed from passing through obstructed - narrowed vessel lumina
Beta thal
Varying shapes
MAHA
Ringed sideronblasts with iron laden mitochondria

50. What do the dense granules contain in platelets
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
ADP and Ca
Extrinsic - I - II - V - VII and X
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 -

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