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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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1. What is the life spance of a platelet?
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
8-10 days
Degrades fibrin mesh and converts C3 to C3a
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

2. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
Tissue
All
MAHA
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

3. What is hemophiliia A
Deficiency in factor VIII
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Glucose and heme - which inhibit ALA synthase
2 to 10

4. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Antigen - antibody
ADP and Ca
GpIIb/IIIa
Down - down - up

5. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
B cells
DIC
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Anti Rh

6. Which pathway and factorrs are tested by the PT coag test
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Folate def
Up - down - nl
Extrinsic - I - II - V - VII and X

7. X linked - dec glutathione inc RBC susceptibility to oxidative stress
5- FU - AZT - hydroxyurea
G6PD
CML
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

8. Sickle cell
Sickle cell anemia
Extravascular hemolysis
Histamine - heparin - and eosinohil chemotactic factors
Lysine for glutamate at position 6

9. What percentage of WBCs are basophils - and What is found in their basophilic granules?
Burkitt lymphoma
Tissue factor converst the VII and VIIa
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Sickle cell

10. Blood type B
40 to 50
B antigena and A antibodes
ADP and Ca
Nl - up - nl

11. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
EBV
Plasma cell
Bernard soulier
Factor V Leiden

12. Serum iron - transferrin - ferritin lab values for hemochromatosis
Uroporphyrinogen decarboxylase
Up - down - up
Decrease EPO
Langerhans cells

13. What does LEAD stand for in lead poisoning?
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Acts to stabilize platelet plug
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 -
Sideroblastic anemia

14. What is the age breakdown for hodgkins
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Bimodal - men>women - except for nodular sclerosing type
Deficiency in factor VIII

15. What happens in betal thal minor?
Orotic aciduria
Howell Jolly bodies
VWD
Beta chain underproduced - asymptomatic - heterozygote

16. Why can newborns with sickle cell be asymptomatic
T(12;21)
TPA - cleavage of fibrin mesh
No
Inc HbF and dec HbS

17. Bite cell
G6PD
Down - down - up
Histamine - heparin - and eosinohil chemotactic factors
Bleeding - malnutrition/absorption - inc demand pregs

18. What does 'Neutrophils Like Making Everything Better' stand for?
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
DIC
Rhogam - Rh antigen immunoglobulin

19. iron deficiency anemia - esophageal web - atrophic glossitis
Inappropriate absolute with inc RBCs and EPO
Ferrocheletase and ALA dehydrogenase
Plummer - vinson syndrome
DIC

20. What is the ddx for aplastic anemia

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21. What does antithrombin do and What activates it?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
CNS and testis
Off center nuclues - clock face chromatin
Inappropriate absolute with inc RBCs and EPO

22. What finding you do you see in patients after splenectomy
B symptoms - fever night sweats - weight loss
Folate def
Howell Jolly bodies
Sickle cell anemia

23. Basophilic nuclear remnants fonud in RBCs
Ankryin - band 4.1 or spectrin
Howell - Jolly bodies - hypo/asplenia
Follicular lymphoma - indolent course
Porphobilinogen deaminase aka uroporphyrinogen I synthase

24. What do you see in vit K def
Megakaryocytes
Eleveated PT - PTT
IFN gama
Diffuse Large b cell lymphoma

25. What is the characteristic finding for MAHA on peripheral smear?
GpIIb/IIIa
Complications of sickle cell anemia
Sickle cell
Schistocytes - helmet cells

26. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Pyruvate kinase def - extravascular
Leukemoid reaction
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
DIC

27. adults - auer rods - inc circulating myeblasts on peripheral smear
Intravascular
Porphobilinogen deaminase aka uroporphyrinogen I synthase
AML
Low O2 in papilla; can also get microhematuria from medullary infarcts

28. Serum iron - transferrin - ferritin lab values for pregs - OCP use
B12/folate def
Hairy cell leukemia
Ticlopidine/clopidogrel
Nl - up - nl

29. What does hairy cell leukemia stain with
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Beta thal
T(12;21)
TRAP (tartrate resistant acid phosphatase

30. Where to T cells arise and mature - and migrate to...
Inc HbF and dec HbS
Lung disease - congenital heart diseaes - and high altitude
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Beta 4

31. What is the difference of presentation of Burkitt in Africa vs the United States
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Imatinib - anti bcr abl
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Africa = Jaw lesion - US = pelvis or abdomen

32. t(15;17)
EBV
M3 AML (acute promyelocytic leukemia)
Inc serum iron - normal TIBC - inc ferratin
DIC - TTP/HUS - traumatic hemolysis

33. Who has more severe disease - HbSS or HbSC
Off center nuclues - clock face chromatin
EBV
Helminth infections major basic protein
HbSS

34. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Inc lymphoctes - and less RS cells
Produce antibody - lots of RER and golgi
EBV
Paroxysmal nocturnal hemoglobinuria

35. Plasma cell neoplasm
Multiple myeloma
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Examples of oxidative stress
Kids= exposure to lead paint - adults = battery - ammunition factory

36. What do platelets interact with to form a hemostatic plug
Fibrinogen
Rhogam - Rh antigen immunoglobulin
Degrades fibrin mesh and converts C3 to C3a
Round densly staining nucleus with a small amount of pale cytoplasm

37. Why does B12 def cause neuro sx?
B12 in fatty acid pathways leads to subacute combined degeneration
Positive osmootic fragility test and splenectomy
Fibrinogen
Rhogam - Rh antigen immunoglobulin

38. What chromosomal translocation is associated with a better prognosis in All
T(12;21)
Varying sizes
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Lymphoma

39. What is the therapy for CML (philly chrom)
Bleeding - malnutrition/absorption - inc demand pregs
Intravascular hemolysis
Imatinib - anti bcr abl
Low in CML

40. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Up - down - up
Valine for glutamate
Sideroblastic anemia
Exposed collagen upon endothelial damage

41. What is the most common hodgkin lymphoma
Inappropriate absolute with inc RBCs and EPO
Nodular sclerosing
Common a few days after oxidative stress in a pt with G6PD
B6 therapy (pyrodixine)

42. What activates the intrinsic pathway?
Collagen - BM - activated platelets
40 to 50
Helminth infections major basic protein
CLL (SLL without the peripheral lymphocytosis

43. What do eosinophils defend against and What do they use to do it?
Hairy cell leukemia
Lacunar in nodular sclerosing variant
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Helminth infections major basic protein

44. What are the likely exposures of kids and adults for lead poisoning
AB - no antibodies
Up - down - up
Kids= exposure to lead paint - adults = battery - ammunition factory
Dec

45. What does increasing heme do to ALA synthase activity
Inappropriate absolute with inc RBCs and EPO
VIII
Blod - tissue - MACS
Dec

46. Fava beans - sufla drugs - infectinos
Lysine for glutamate at position 6
Lysosomes
Examples of oxidative stress
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

47. What does ectopic EPO produce
Inappropriate absolute with inc RBCs and EPO
Inc vasodiltion - inc perm - inc pain
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Howell Jolly bodies

48. What is the therapy for the M3 variant?
Beta 4
All trans retinoic acid
CLL (SLL without the peripheral lymphocytosis
Fibrinogen

49. What vitamin is a cofactor for the first step of heme synthesis
Uroporphyrin (tea colored urine)
VWD
B6
Histamine - heparin - and eosinohil chemotactic factors

50. What is the tx for vWD
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Inc lymphoctes - and less RS cells
Langerhans cell histiocytosis
Varying shapes