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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 pathogenesis of aplastic anemia with kidney disease
Decrease EPO
Complications of sickle cell anemia
Ticlopidine/clopidogrel
Tissue factor converst the VII and VIIa

2. philadelphia chromosome - blood looks like marrow
Ewing sarcoma
Produce antibody - lots of RER and golgi
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
CML

3. Which immunoglobulin is involved in warm agglutination?
Burkitt lymphoma
IgG - Warm is GREAT
Up - down - nl
Tissue

4. Plasma cell neoplasm
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Blood
Multiple myeloma
Def in factor IX

5. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Factor V Leiden
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Multiple myeloma
Sickle cell

6. How does vWD cause elevated PTT?
VWF carries/protects factor VIII
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Blod - tissue - MACS

7. Is G6PD intravascular or extravascular
Idiopathic thrombocytopenia (ITP)
Intravascular
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Nl PT - elevated PTT - intrinsic pathway defect

8. What substance is Fe added to to yield heme
T(12;21)
Protoporphyrin
Examples of oxidative stress
Hereditary spherocytosis - autoimmune hemolysis

9. Where do B cells arise from - mature - and migrate to...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Helminth infections major basic protein
Intrinsic - all factors except - VII - XIII
Anemia of chronic disease - Aplastic anemia - kidney disease

10. Eosinophils are highly phagocytic For what kind of complex?
Antigen - antibody
40 to 75
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Acts to stabilize platelet plug

11. t(11;14)
Blood
40 to 50
Mantle cell lymphoma
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

12. What do the platelets bind? What is the step called
B12 in fatty acid pathways leads to subacute combined degeneration
AB - no antibodies
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
They bind vWF via GpIb

13. What converts plasminogen to plasm and What does plasmin do?
TPA - cleavage of fibrin mesh
Histamine - heparin - and eosinohil chemotactic factors
Def in factor IX
Blasts > 5%

14. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Helps platelts adhere to endothelium
Leukemoid reaction
Bite cells and Heinz bodies
Heinz bodies - seen in alpha thal and G6PD

15. What does ectopic EPO produce
Inappropriate absolute with inc RBCs and EPO
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Glycine and succinyl - coa
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

16. Where are mast cells found
1 to 6
Mantle cell lymphoma
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Tissue

17. How do platelet disorders present?
Folate def
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Aplastic anemia - pancytopenia

18. What is the main source of energy in RBCs
Histaminase and arylsulfatase
Hb Barts - gamma4 in defect in all 4 alpha genes
90% anearobically from glucose to lactate - 10% from HMP shunt
Rhogam - Rh antigen immunoglobulin

19. What is the Ddx for a normocytic - normochromic anemia?
B12 def
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

20. What is the philadelphia chromosome
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
T(9;22) bcr abl
Histaminase and arylsulfatase
ATIII def

21. Drug that inhbits the GpIIb/IIIa directly
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Sideroblastic anemia
T(9;22) bcr abl
Abciximab

22. What do labs show in DIC?
Diffuse Large b cell lymphoma
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Sheets of lymphocytes interspersed with macs
All

23. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
VWD
Bleeding - malnutrition/absorption - inc demand pregs
Pos
Ewing sarcoma

24. What is the therapy for the M3 variant?
All trans retinoic acid
Examples of oxidative stress
No lytic bone lesions in WM
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

25. Ringed sideroblasts
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Sideroblastic anemia
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 -
B cells

26. Does CML have a JAK2 mut
Examples of oxidative stress
No
Positive osmootic fragility test and splenectomy
Protein C or S deficiency

27. What are the azuraphilic granules in PMNs
Dec plasma volume
Helminth infections major basic protein
Lysosomes
Nodular sclerosing

28. What is contained within the azurophilic granules of PMNs
All
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
DIC
Pyruvate kinase def - extravascular

29. Conditions of defective heme synthesis leading to accumulation of heme precurors
Porphyrias
VWF carries/protects factor VIII
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Lymphoma

30. What virus is associated with Burkitt lymphoma
Abciximab
Helps platelts adhere to endothelium
Inc HbA2 on electrophoresis
EBV

31. Elderly - mature b cell tumor with filamentous - hairlike projections
Hairy cell leukemia
5- FU - AZT - hydroxyurea
AB - no antibodies
All

32. What is the tx for sickle cell
T(9;22) bcr abl
Lacunar in nodular sclerosing variant
Hydroxyurea - bone marrow transplant
Hairy cell leukemia

33. What are the neuro sx of B12 def?
Glycine and succinyl - coa
Birbeck granules
Induces differentiation of myeloblasts
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

34. What do platelets interact with to form a hemostatic plug
Fibrinogen
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Lead - and EtOH
Sickle cell

35. What is the Ddx for for a macrocytic anemia
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Collagen - BM - activated platelets
Mantle cell lymphoma

36. What does LEAD stand for in lead poisoning?
Folate def
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 -
Gp1b
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

37. What vitamin is a cofactor for the first step of heme synthesis
B6
All trans retinoic acid
Inappropriate absolute with inc RBCs and EPO
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

38. What do you see a starry sky appearance in Burkitt
40 to 75
20 to 40
Sheets of lymphocytes interspersed with macs
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

39. What does decreasing heme do to ALA synthase activity?
Down - down - up
Bone marrow infiltration - myelofibrosis
Inc
HbSS

40. What are the presenting symptoms of acute intermittent porphyria
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Essential thrombocytosis
Antigen - antibody
Mantle cell - older males

41. Acanthocyte (spur cell)
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Liver disease - abetalipoproteinemia - acntho = spiny
Reed - sternberg cells
Epoxide reductase - warfarin inhibits

42. What substance prevents mast cells degranulation?
Cromolyn sodium
Bone marrow infiltration - myelofibrosis
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

43. What signal activates MACS
Malaria - Babesia
IFN gama
GpIIb/IIIa
Collagen - BM - activated platelets

44. What is the therapy for CML (philly chrom)
Imatinib - anti bcr abl
Schistocytes - helmet cells
90% anearobically from glucose to lactate - 10% from HMP shunt
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

45. What do you see in peripheral smear in a pt with G6PD?
Orotic aciduria
Bite cells and Heinz bodies
EBV
Ankryin - band 4.1 or spectrin

46. bcl -2 t(14;18) adults
All
Follicular lymphoma - indolent course
Kids= exposure to lead paint - adults = battery - ammunition factory
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

47. What are the variants of the RS cells
A antigen and B antibodies
B symptoms - fever night sweats - weight loss
Lacunar in nodular sclerosing variant
Low O2 in papilla; can also get microhematuria from medullary infarcts

48. What is the life span of a normal RBC
Multiple myeloma
Langerhans cells
120 days
5- FU - AZT - hydroxyurea

49. What state is commonly associated with nonHod lymphoma
Gp1b
HIV or immunosupression
TRAP (tartrate resistant acid phosphatase
Signs and sx of aplastic anemia

50. What is the most common hodgkin lymphoma
Decrease EPO
Beta 4
Factor V resistant to activated protein C's inhibition
Nodular sclerosing