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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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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. What is the difference of presentation of Burkitt in Africa vs the United States
Alpha thal - asian and african american
Africa = Jaw lesion - US = pelvis or abdomen
Inc RBC - dec O2 sat - inc EPO
Inc serum iron - normal TIBC - inc ferratin

2. What are the anti aggregation factors?
Langerhans cell histiocytosis
PGI2 - NO inc blood flow - dec platelet aggregation
Mycosis fundgoides/Sezary syndrome
VWF carries/protects factor VIII

3. What activates the fibrinolytic pathway?
Ewing sarcoma
Her next Rh+ fetus
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Tissue

4. Which substrates begin the heme synthesis pathway
Yes - via MHC II
Glycine and succinyl - coa
Abciximab
Hereditary spherocytosis - G6PD - sickle cell

5. X linked - dec glutathione inc RBC susceptibility to oxidative stress
TPA - cleavage of fibrin mesh
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
CD15 and CD30 pos
G6PD

6. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
DIC
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Anemia of chronic disease - Aplastic anemia - kidney disease
...

7. What do platelets interact with to form a hemostatic plug
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Fibrinogen
Idiopathic thrombocytopenia (ITP)
Yes - via MHC II

8. What is the treatment for acute intermittent porphyria
Both alpha and beta thal
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Hb Barts - gamma4 in defect in all 4 alpha genes
Glucose and heme - which inhibit ALA synthase

9. Deficiency in vWF

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10. When is the peak incidence for nonHod lymphoma
20 to 40
Blod - tissue - MACS
IgG - Warm is GREAT
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

11. drug that inhibits ADP induced expression of GpIIb/IIIa
Von Willebrand's disease
Bleeding - malnutrition/absorption - inc demand pregs
Blistering cutaneous photosens - most common porphyria
Ticlopidine/clopidogrel

12. Mutation in 3' untranslated region associated with venous clots
Prothrombin gene mutation
Pyruvate kinase def - extravascular
Def in factor IX
Howell Jolly bodies

13. What is the coombs test results in AIHA?
Lacunar in nodular sclerosing variant
Porphyrias
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Pos

14. Which pathway and factors are tested in the PTT test
1 to 6
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Intrinsic - all factors except - VII - XIII
No antigen - both antibodies

15. What is the most common hodgkin lymphoma
90% anearobically from glucose to lactate - 10% from HMP shunt
Anemia of chronic disease - Aplastic anemia - kidney disease
Intravascular
Nodular sclerosing

16. What causes the jaundice in extravascular hemolysis
MHC II and Fc receptors
PGI2 - NO inc blood flow - dec platelet aggregation
Ewing sarcoma
Inc UCB

17. What are the extrinsic hemolytic normocytic anemias?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
MAHA
...
Direct coombs - agglutinate if RBCs are coated with Ig

18. Spherocyte
Imatinib - anti bcr abl
Beta thal
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Hereditary spherocytosis - autoimmune hemolysis

19. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Inc lymphoctes - and less RS cells
Fe def - thal - pb poison - sideroblastic anemia
Complications of sickle cell anemia

20. Acanthocyte (spur cell)
Liver disease - abetalipoproteinemia - acntho = spiny
No platelet clumping
Prothrombin gene mutation
Nodular sclerosing

21. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Lysine for glutamate at position 6
Signs and sx of aplastic anemia
Uroporphyrin (tea colored urine)

22. poikilocytosis
Aspirin
Yes - via MHC II
Varying shapes
Bleeding - malnutrition/absorption - inc demand pregs

23. What is the life spance of a platelet?
IgG - Warm is GREAT
Reed - sternberg cells
Hereditary spherocytosis - autoimmune hemolysis
8-10 days

24. What does bradykinin do?
MAHA
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Leukemia
Inc vasodiltion - inc perm - inc pain

25. hypocellular bone marrown tih fatty infiltration
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Aplastic anemia - pancytopenia
Lung disease - congenital heart diseaes - and high altitude
Porphobilinogen deaminase aka uroporphyrinogen I synthase

26. What percentage if WBCs are polys?
Her next Rh+ fetus
TRAP (tartrate resistant acid phosphatase
40 to 75
Factor V resistant to activated protein C's inhibition

27. What do platelets release necessary for coagulation cascaed?
Exposed collagen upon endothelial damage
Uroporphyrinogen decarboxylase
ADP and Ca
Protein C or S deficiency

28. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Imatinib - anti bcr abl
Aspirin
Hodgkin
AB - no antibodies

29. Why does B12 def cause neuro sx?
B12 in fatty acid pathways leads to subacute combined degeneration
VIII
Valine for glutamate
Low in CML

30. What does CRAB stand for in multiple myeloma
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Macrohemorrhage - hemarthroses - easy bruising
TPA - cleavage of fibrin mesh

31. What is the age group most commonly affected by multiple myeloma
Plummer - vinson syndrome
40 to 50
B12 def
Down - up - down

32. What substance is Fe added to to yield heme
Sickle cell anemia
Contiguous
Myelofibrosis (marow is crying being its fibrosed'
Protoporphyrin

33. What portion of IgE can mast cells bind
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Abciximab
Fc
Collagen - BM - activated platelets

34. Can B cells function as APCs?
Rhogam - Rh antigen immunoglobulin
Yes - via MHC II
ADP and Ca
Langerhans cells

35. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Megakaryocytes
Signs and sx of aplastic anemia
Fibrinogen
Down - down - up

36. What does decreasing heme do to ALA synthase activity?
DIC - TTP/HUS - traumatic hemolysis
Mycosis fundgoides/Sezary syndrome
Inc
Von Willebrand's disease

37. What substances are released in mast cell degranulation
Leukemoid reaction
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Inducers of primary antibody response
Histamine - heparin - and eosinohil chemotactic factors

38. Elderly - mature b cell tumor with filamentous - hairlike projections
Inc HbF and dec HbS
Hairy cell leukemia
...
Produce antibody - lots of RER and golgi

39. What is the age breakdown for hodgkins
Paroxysmal nocturnal hemoglobinuria
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
B12 in fatty acid pathways leads to subacute combined degeneration
Bimodal - men>women - except for nodular sclerosing type

40. What is the effected enzyme in acute intermittment porphyria?
Varying sizes
Follicular lymphoma - indolent course
Positive osmootic fragility test and splenectomy
Porphobilinogen deaminase aka uroporphyrinogen I synthase

41. How are plasma cells characterized?
Off center nuclues - clock face chromatin
Reed - sternberg cells
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

42. What is the mutation in HbS
Valine for glutamate
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Schistocytes - helmet cells
Bone marrow - thymus - blood (80% of circulating lymphos are T)

43. What is the characteristic histologic finding in Hodgkin Lymphoma
Reed - sternberg cells
VIII
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Peroxidase

44. t(15;17)
Protein C or S deficiency
T(9;22) bcr abl
M3 AML (acute promyelocytic leukemia)
DIC

45. Where do B cells arise from - mature - and migrate to...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Fc
CML to AML or All
Glucose and heme - which inhibit ALA synthase

46. Sickle cell
Hereditary elloptocytosis
Sickle cell anemia
PGI2 - NO inc blood flow - dec platelet aggregation
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

47. What does Vit K deficiency cause?
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
IgG - Warm is GREAT
Dec synthesis of factors 1972 - protein C/S
No antigen - both antibodies

48. What are the presenting symptoms of acute intermittent porphyria
Inc serum iron - normal TIBC - inc ferratin
Extravascular hemolysis
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
No

49. What percentage of WBCs are monocytes?
G6PD
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
No platelet clumping
2 to 10

50. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
DIC
Leukemoid reaction
Langerhans cells
Lacunar in nodular sclerosing variant