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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. Is HS extravascular or intravascular?
Langerhans cells
Examples of oxidative stress
Mantle cell lymphoma
Extravascular

2. What causes the physiologic chloride shift and What does the chloride shift do?
8-10 days
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Protoporphyrin (blood)
Plummer - vinson syndrome

3. what proteins can be defective in HS?
Myelofibrosis (marow is crying being its fibrosed'
Ankryin - band 4.1 or spectrin
All trans retinoic acid
A antigen and B antibodies

4. adults - auer rods - inc circulating myeblasts on peripheral smear
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
AML
5- FU - AZT - hydroxyurea
Uroporphyrin (tea colored urine)

5. What virus can cause an aplastic crisis in pts with HS?
Parvovirus
Inducers of primary antibody response
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Cromolyn sodium

6. What is the main source of energy in RBCs
Kids= exposure to lead paint - adults = battery - ammunition factory
90% anearobically from glucose to lactate - 10% from HMP shunt
Fc
HIV or immunosupression

7. What converts plasminogen to plasm and What does plasmin do?
Ticlopidine/clopidogrel
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
TPA - cleavage of fibrin mesh
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

8. What does the large SA:volume ratio in RBCs help facilitate?
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Inc large vWF multimers - inc platelet aggregation and thrombosis
Easy gas exchange
Degrades fibrin mesh and converts C3 to C3a

9. What is the life spance of a platelet?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Lung disease - congenital heart diseaes - and high altitude
Inappropriate absolute with inc RBCs and EPO
8-10 days

10. Where do B cells arise from - mature - and migrate to...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Fibrinogen bind GpIIb/IIIa and links platelts
Multiple myeloma
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

11. Serum iron - transferrin - ferritin lab values for iron def anemia
Schistocytes and inc LDH
Down - up - down
Fibrinogen
Tissue factor converst the VII and VIIa

12. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
B12 def
Reed - sternberg cells
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Paroxysmal nocturnal hemoglobinuria

13. Can B cells function as APCs?
DIC
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Ticlopidine/clopidogrel
Yes - via MHC II

14. What are the azuraphilic granules in PMNs
Ticlopidine/clopidogrel
Bite cells and Heinz bodies
Up - down - up
Lysosomes

15. Is G6PD intravascular or extravascular
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Intravascular
Abciximab
Ferrocheletase and ALA dehydrogenase

16. Where are mast cells found
Leukemia
Common a few days after oxidative stress in a pt with G6PD
Tissue
Induces differentiation of myeloblasts

17. t(8;14) c - myc gene
Inc
Burkitt lymphoma
Lung disease - congenital heart diseaes - and high altitude
Dec synthesis of factors 1972 - protein C/S

18. What finding you do you see in patients after splenectomy
Howell Jolly bodies
Folate def
Abciximab
B12/folate def

19. Fibrotic obliteration of bone marow with teardrop cells

20. What does decreasing heme do to ALA synthase activity?
Lung disease - congenital heart diseaes - and high altitude
Hodgkin
20 to 40
Inc

21. Who are the professional APCs?
VWF and fibrinogen
Alpha thal - asian and african american
Dendritic cells?
Easy gas exchange

22. What is the Ddx for a normocytic - normochromic anemia?
Essential thrombocytosis
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Inc HbF and dec HbS

23. What is appropriate absolyte polycythemia associated with
Paraprotein spike - monoclonal protein
Complications of sickle cell anemia
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Lung disease - congenital heart diseaes - and high altitude

24. How are plasma cells characterized?
Complications of sickle cell anemia
Inc
TPA - cleavage of fibrin mesh
Off center nuclues - clock face chromatin

25. What percentage of WBCs are monocytes?
Imatinib - anti bcr abl
CNS and testis
DIC - TTP/HUS - traumatic hemolysis
2 to 10

26. Which pathway and factors are tested in the PTT test
Blasts > 5%
Intrinsic - all factors except - VII - XIII
B6
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

27. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
A antigen and B antibodies
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Lymphoma

28. What causes hydrops fetalis
Degrades fibrin mesh and converts C3 to C3a
Hb Barts - gamma4 in defect in all 4 alpha genes
Inducers of primary antibody response
CML

29. What do the iron studies show in sideroblastic anemia
Sideroblastic anemia
Inc serum iron - normal TIBC - inc ferratin
T(12;21)
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

30. What do the platelets bind? What is the step called
Polycythemia vera
They bind vWF via GpIb
Bite cells and Heinz bodies
Spleen

31. What does bradykinin do?
A antigen and B antibodies
Inc vasodiltion - inc perm - inc pain
Sickle cell anemia
GpIIb/IIIa

32. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Leukemoid reaction
Down - up - down
M3 AML (acute promyelocytic leukemia)

33. Which substrates begin the heme synthesis pathway
IgG - Warm is GREAT
Rhogam - Rh antigen immunoglobulin
Nodular sclerosing
Glycine and succinyl - coa

34. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Mycosis fundgoides/Sezary syndrome
Petechiae
Beta chain underproduced - asymptomatic - heterozygote
Folate def

35. tennis rackets on EM
Birbeck granules
All
Down - up - down
Off center nuclues - clock face chromatin

36. What is the treatment to prevent a woman from forming anti Rh antibody?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Tissue
T(12;21)
Rhogam - Rh antigen immunoglobulin

37. What the alpha granules contain in platelets?
Anti Rh
VWF and fibrinogen
Imatinib - anti bcr abl
Hodgkin

38. What do you see a starry sky appearance in Burkitt
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
...
HIV or immunosupression
Sheets of lymphocytes interspersed with macs

39. What is the therapy for the M3 variant?
All trans retinoic acid
TPA - cleavage of fibrin mesh
Extrinsic - I - II - V - VII and X
Varying sizes

40. What are some causes of warm agglutinin autoimmune hemolytic anemia?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Burkitt lymphoma
SLE - CLL - alpha methyldopa
ATIII def

41. What vitamin is a cofactor for the first step of heme synthesis
Myelofibrosis (marow is crying being its fibrosed'
CNS and testis
B6
Inc UCB

42. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Histaminase and arylsulfatase
B6 therapy (pyrodixine)
B12/folate def
Inc vasodiltion - inc perm - inc pain

43. What indicated a good prognosis in Hodgkin lymphoma?
Inc lymphoctes - and less RS cells
IgG - Warm is GREAT
Adult T cell lymphoma - presents with cutaneous lesions
Varying sizes

44. What is the result of thrombocytopenia or platelet dysfunction?
Deficiency in factor VIII
Petechiae
Anti Rh
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

45. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
No
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Sickle cell
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

46. In hemophilia A or B What do you see in the coag tests
Nl PT - elevated PTT - intrinsic pathway defect
Hereditary spherocytosis - G6PD - sickle cell
Kids= exposure to lead paint - adults = battery - ammunition factory
Inappropriate absolute with inc RBCs and EPO

47. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Multiple myeloma
PGI2 - NO inc blood flow - dec platelet aggregation
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
No lytic bone lesions in WM

48. What is the life span of a normal RBC
120 days
Plummer - vinson syndrome
20 to 40
Mycosis fundgoides/Sezary syndrome

49. What are the main associations with multiple myeloma?
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Myelofibrosis (marow is crying being its fibrosed'
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
No platelet clumping

50. What is the characteristic spread of Hodgkin Lymphoma
Contiguous
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Thrombotic thrombocytopenic purpura
CNS and testis