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Hemeonc

Instructions:

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. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Degrades fibrin mesh and converts C3 to C3a
Blod - tissue - MACS
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
VWD

2. What is the effect of ACE on bradykinin
Lysine for glutamate at position 6
Inactivates it
Extrinsic - I - II - V - VII and X
Hairy cell leukemia

3. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
MHC II and Fc receptors
Howell - Jolly bodies - hypo/asplenia
DIC - TTP/HUS - traumatic hemolysis

4. What do platelets release necessary for coagulation cascaed?
Induces differentiation of myeloblasts
Essential thrombocytosis
Nodular sclerosing
ADP and Ca

5. HTLV-1
Blistering cutaneous photosens - most common porphyria
Beta thal
Adult T cell lymphoma - presents with cutaneous lesions
B12/folate def

6. t(15;17)
Plasma cell
Down - up - down
Protoporphyrin (blood)
M3 AML (acute promyelocytic leukemia)

7. What substances are released in mast cell degranulation
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Inc RBC - dec O2 sat - inc EPO
Histamine - heparin - and eosinohil chemotactic factors
Hereditary elloptocytosis

8. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Nl - up - nl
Hereditary spherocytosis
ADP and Ca
Both alpha and beta thal

9. What state is commonly associated with nonHod lymphoma
Extravascular hemolysis
Bernard soulier
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
HIV or immunosupression

10. What is a blast crisis
Beta 4
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Down - down - up
CML to AML or All

11. After an injury - What does vWF bind to begin platelet plug formation?
Exposed collagen upon endothelial damage
VWD
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Factor V Leiden

12. What is the clinical picture of hemophilia A or B
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Macrohemorrhage - hemarthroses - easy bruising
Acts to stabilize platelet plug
Yes - via MHC II

13. What do plasma cells do
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Produce antibody - lots of RER and golgi
Hereditary elloptocytosis
Inappropriate absolute with inc RBCs and EPO

14. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Fibrinogen
Leukemoid reaction
Incactivates II - VII - IX - X - XI - XII
ATIII def

15. What is the effected enzyme in acute intermittment porphyria?
DIC
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Positive osmootic fragility test and splenectomy
Malaria - Babesia

16. Serum iron - transferrin - ferritin lab values for hemochromatosis
Lacunar in nodular sclerosing variant
Orotic aciduria
Up - down - up
HbSS

17. Deficiency in GpIb
Inc HbA2 on electrophoresis
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Bernard soulier

18. What the alpha granules contain in platelets?
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Paroxysmal nocturnal hemoglobinuria
VWF and fibrinogen
Folate def

19. What is the receptor for fibrinogen?
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
GpIIb/IIIa
Ferrocheletase and ALA dehydrogenase
Rhogam - Rh antigen immunoglobulin

20. What do you see on peripheral smear with sideroblastic anemia
Dec plasma volume
Ringed sideronblasts with iron laden mitochondria
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
CD15 and CD30 pos

21. What do eosinophils defend against and What do they use to do it?
Nl - up - nl
Hodgkin
Helminth infections major basic protein
Leukemia

22. What is the general pathology of a macrocytic anemia?
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Up - down - up
CML to AML or All
Hereditary spherocytosis

23. What does antithrombin do and What activates it?
Varying shapes
Intrinsic - all factors except - VII - XIII
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
HIV or immunosupression

24. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Africa = Jaw lesion - US = pelvis or abdomen
90% anearobically from glucose to lactate - 10% from HMP shunt
Leukemoid reaction
Epoxide reductase - warfarin inhibits

25. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Glanzmann's throbmasthenia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Ewing sarcoma
Abciximab

26. adults - auer rods - inc circulating myeblasts on peripheral smear
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Polycythemia vera
AML
Inc lymphoctes - and less RS cells

27. What is the main source of energy in RBCs
Leukemoid reaction
90% anearobically from glucose to lactate - 10% from HMP shunt
T(9;22) bcr abl
Ringed sideronblasts with iron laden mitochondria

28. What activates protein C and What does activated protein C do?
CML to AML or All
Extravascular
TXA2 - dec blood flow - inc platelet aggregation
Protein S - cleaves and invactivates Va and VIIIa

29. What causes the physiologic chloride shift and What does the chloride shift do?
Reed - sternberg cells
Bone marrow infiltration - myelofibrosis
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Pyruvate kinase def - extravascular

30. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Idiopathic thrombocytopenia (ITP)
PGI2 - NO inc blood flow - dec platelet aggregation
Inc lymphoctes - and less RS cells
Heinz bodies - seen in alpha thal and G6PD

31. Bite cell
Abciximab
ADP and Ca
G6PD
Easy gas exchange

32. CD5+ - poor prognosis - t(11;14)
Antigen - antibody
Langerhans cell histiocytosis
Fe def - thal - pb poison - sideroblastic anemia
Mantle cell - older males

33. Ddx for microcytic anemia
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Fe def - thal - pb poison - sideroblastic anemia
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Aplastic anemia - pancytopenia

34. Target cell

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35. Where does All spread
CNS and testis
Anemia of chronic disease - Aplastic anemia - kidney disease
They bind vWF via GpIb
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

36. What causes hydrops fetalis
All trans retinoic acid
PGI2 - NO inc blood flow - dec platelet aggregation
Hb Barts - gamma4 in defect in all 4 alpha genes
Aspirin

37. universal donor
EBV
No antigen - both antibodies
Inactivates it
120 days

38. What is the characteristic histologic finding in Hodgkin Lymphoma
Factor V resistant to activated protein C's inhibition
Inc vasodiltion - inc perm - inc pain
Inc serum iron - normal TIBC - inc ferratin
Reed - sternberg cells

39. What is the aggregation phase of ppf?
Aplastic anemia - pancytopenia
Fibrinogen bind GpIIb/IIIa and links platelts
Indirect coombs - agglutinate if serum anti RBC surface Ig
Heinz bodies - seen in alpha thal and G6PD

40. What causes the jaundice in extravascular hemolysis
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
HbSS
8-10 days
Inc UCB

41. What do the labs show for TTP?
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Schistocytes and inc LDH
T(12;21)
MGUS - monoclonal gammopathy of undetermined significance

42. What are the pro aggregation factors?
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Up - down - up
TXA2 - dec blood flow - inc platelet aggregation
Glucose and heme - which inhibit ALA synthase

43. What are the labs and tx for HS?
Hairy cell leukemia
Positive osmootic fragility test and splenectomy
Malaria - Babesia
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

44. What are the presenting symptoms of porphyria cutanea tarda
Blistering cutaneous photosens - most common porphyria
Fc
Ringed sideronblasts with iron laden mitochondria
Mycosis fundgoides/Sezary syndrome

45. normal RBCs added to patient's serum
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
B symptoms - fever night sweats - weight loss
Indirect coombs - agglutinate if serum anti RBC surface Ig

46. Where are 1/3 of platelets stored
Howell - Jolly bodies - hypo/asplenia
Reed - sternberg cells
Spleen
G6PD

47. iron deficiency anemia - esophageal web - atrophic glossitis
Beta chain underproduced - asymptomatic - heterozygote
Plummer - vinson syndrome
Indirect coombs - agglutinate if serum anti RBC surface Ig
Langerhans cells

48. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Pyruvate kinase def - extravascular
B12 def
Down - up - down
Plummer - vinson syndrome

49. Fava beans - sufla drugs - infectinos
Examples of oxidative stress
A antigen and B antibodies
...
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

50. anisocytosis
8-10 days
Varying sizes
HIV or immunosupression
Def in factor IX