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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 substance prevents mast cells degranulation?
40 to 75
CD15 and CD30 pos
Cromolyn sodium
GpIIb/IIIa

2. What does ADP do?
Bone marrow - thymus - blood (80% of circulating lymphos are T)
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Helps platelts adhere to endothelium
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

3. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Up - down - nl
Factor V resistant to activated protein C's inhibition
Hereditary spherocytosis - G6PD - sickle cell
Intravascular hemolysis

4. What is the treatment for lead poising?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Hydroxyurea - bone marrow transplant
Intravascular
5- FU - AZT - hydroxyurea

5. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Folate def
Intravascular hemolysis
Decrease EPO
Hydroxyurea - bone marrow transplant

6. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Aplastic anemia - pancytopenia
M3 AML (acute promyelocytic leukemia)
Factor V Leiden
Inc large vWF multimers - inc platelet aggregation and thrombosis

7. What does antithrombin do and What activates it?
Inactivates it
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
B6 therapy (pyrodixine)
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

8. How does the therapy for M3 vairant work?
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Induces differentiation of myeloblasts
CLL (SLL without the peripheral lymphocytosis
Blistering cutaneous photosens - most common porphyria

9. What is the affected enzyme in acute intermittment porphyria
Porphobilinogen - delta ALA - uroporphyrin
They bind vWF via GpIb
G6PD
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

10. What do you see in vit K def
Kids= exposure to lead paint - adults = battery - ammunition factory
Eleveated PT - PTT
Positive osmootic fragility test and splenectomy
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

11. What finding you do you see in patients after splenectomy
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
No
Exposed collagen upon endothelial damage
Howell Jolly bodies

12. What virus can cause an aplastic crisis in pts with HS?
Sideroblastic anemia
Parvovirus
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Plasma cell

13. adults - auer rods - inc circulating myeblasts on peripheral smear
Aspirin
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
AML

14. What is monoclonal expansion without symptoms associated with multiple myeloma?
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Protein C or S deficiency
Helminth infections major basic protein
MGUS - monoclonal gammopathy of undetermined significance

15. t(11:22)
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Varying sizes
Ewing sarcoma

16. What is the tx for sickle cell
Def in factor IX
Helminth infections major basic protein
MGUS - monoclonal gammopathy of undetermined significance
Hydroxyurea - bone marrow transplant

17. What is the life span of a normal RBC
120 days
Von Willebrand's disease
Helps platelts adhere to endothelium
All

18. Where to T cells arise and mature - and migrate to...
20 to 40
Alpha thal - asian and african american
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Bone marrow - thymus - blood (80% of circulating lymphos are T)

19. What are the labs and tx for HS?
Positive osmootic fragility test and splenectomy
Dendritic cells?
Low in CML
Anemia of chronic disease - Aplastic anemia - kidney disease

20. What is the treatment for acute intermittent porphyria
Varying sizes
Contiguous
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Glucose and heme - which inhibit ALA synthase

21. What is the pathogenesis of TTP?
Up - down - up
Direct coombs - agglutinate if RBCs are coated with Ig
Inc large vWF multimers - inc platelet aggregation and thrombosis
Acts to stabilize platelet plug

22. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
20 to 40
VWF carries/protects factor VIII
M3 AML (acute promyelocytic leukemia)
All

23. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Multiple myeloma
Splicing sites and promotor sequences
Langerhans cells
Idiopathic thrombocytopenia (ITP)

24. What begins the extrinsic pathway?
Fc
Exposed collagen upon endothelial damage
DIC
Tissue factor converst the VII and VIIa

25. Why does B12 def cause neuro sx?
Sideroblastic anemia
20 to 40
Uroporphyrinogen decarboxylase
B12 in fatty acid pathways leads to subacute combined degeneration

26. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
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 -
...
Kids= exposure to lead paint - adults = battery - ammunition factory
Intravascular hemolysis

27. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Inducers of primary antibody response
Varying shapes
Rhogam - Rh antigen immunoglobulin
VWD

28. Target cell

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29. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
B12 in fatty acid pathways leads to subacute combined degeneration
Histaminase and arylsulfatase
Collagen - BM - activated platelets
Easy gas exchange

30. What does decreasing heme do to ALA synthase activity?
Bimodal - men>women - except for nodular sclerosing type
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Fibrinogen bind GpIIb/IIIa and links platelts
Inc

31. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Epoxide reductase - warfarin inhibits
ATIII def
Fibrinogen
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

32. What the alpha granules contain in platelets?
Lymphoma
Inducers of primary antibody response
Varying sizes
VWF and fibrinogen

33. bcl -2 t(14;18) adults
IgG - Warm is GREAT
Tissue
Follicular lymphoma - indolent course
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

34. Fibrotic obliteration of bone marow with teardrop cells

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35. What is the effected enzyme in acute intermittment porphyria?
Howell - Jolly bodies - hypo/asplenia
Inc RBC - dec O2 sat - inc EPO
Acts to stabilize platelet plug
Porphobilinogen deaminase aka uroporphyrinogen I synthase

36. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Dendritic cells?
Sideroblastic anemia
Lysosomes
Signs and sx of aplastic anemia

37. What virus is associated with Burkitt lymphoma
EBV
2 to 10
Hereditary elloptocytosis
B antigena and A antibodes

38. Deficiency in vWF

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39. universal recipient
AB - no antibodies
Varying shapes
Varying sizes
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

40. What percentage if WBCs are polys?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
40 to 75
ADP and Ca

41. What is HbH
Both alpha and beta thal
Beta 4
Dec synthesis of factors 1972 - protein C/S
CML

42. What do labs show in ITP?
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Protein S - cleaves and invactivates Va and VIIIa
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Extravascular hemolysis

43. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Bite cells and Heinz bodies
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Nl - up - nl
IFN gama

44. What does CRAB stand for in multiple myeloma
Histaminase and arylsulfatase
Orotic aciduria
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

45. Wilm's tumor - RCC - HCC - hydronephrosis
Bernard soulier
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
...
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

46. Sickle cell
Anemia of chronic disease - Aplastic anemia - kidney disease
Protein C or S deficiency
No lytic bone lesions in WM
Sickle cell anemia

47. What CD molecules are on RS cells
Follicular lymphoma - indolent course
Aplastic anemia - pancytopenia
CD15 and CD30 pos
Exposed collagen upon endothelial damage

48. HTLV-1
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
All
Hairy cell leukemia
Adult T cell lymphoma - presents with cutaneous lesions

49. What is a blast crisis
CML to AML or All
Thrombotic thrombocytopenic purpura
Langerhans cell histiocytosis
Bite cells and Heinz bodies

50. What do they express on their surface?
5- FU - AZT - hydroxyurea
MHC II and Fc receptors
Bone marrow infiltration - myelofibrosis
MAHA

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