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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 are the four levels of alpha thal?
Folate def
EBV
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Peroxidase

2. How does vWD cause elevated PTT?
VWF carries/protects factor VIII
Schistocytes - helmet cells
Langerhans cells
Inc

3. What activates protein C and What does activated protein C do?
120 days
Dec synthesis of factors 1972 - protein C/S
Protein S - cleaves and invactivates Va and VIIIa
Her next Rh+ fetus

4. After an injury - What does vWF bind to begin platelet plug formation?
Bone marrow infiltration - myelofibrosis
Bite cells and Heinz bodies
Exposed collagen upon endothelial damage
Helps platelts adhere to endothelium

5. CD5+ - poor prognosis - t(11;14)
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
EBV
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Mantle cell - older males

6. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Blod - tissue - MACS
Protein C or S deficiency

7. t(11;14)
Kids= exposure to lead paint - adults = battery - ammunition factory
T(9;22) bcr abl
Orotic aciduria
Mantle cell lymphoma

8. What is appropriate absolyte polycythemia associated with
Lung disease - congenital heart diseaes - and high altitude
Bone marrow infiltration - myelofibrosis
Sickle cell anemia
120 days

9. What is a blast crisis
No antigen - both antibodies
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
CML to AML or All
Lacunar in nodular sclerosing variant

10. universal donor
AML
No antigen - both antibodies
Mantle cell lymphoma
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

11. What does LEAD stand for in lead poisoning?
Inactivates it
Hb Barts - gamma4 in defect in all 4 alpha genes
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 -
Paraprotein spike - monoclonal protein

12. Where do you see hypersegmented polys?
Paroxysmal nocturnal hemoglobinuria
Off center nuclues - clock face chromatin
B12/folate def
Down - up - down

13. Target cell

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14. tennis rackets on EM
120 days
Inc serum iron - normal TIBC - inc ferratin
Multiple myeloma
Birbeck granules

15. What does STOP Making New Thrombi stand for
Anti Rh
Easy gas exchange
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Eleveated PT - PTT

16. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
G6PD
5- FU - AZT - hydroxyurea
Alpha thal - asian and african american

17. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Inc UCB
Thrombotic thrombocytopenic purpura
Yes - via MHC II
Paroxysmal nocturnal hemoglobinuria

18. Basophilica stippling
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
A antigen and B antibodies
Protoporphyrin
Dec

19. Conditions of defective heme synthesis leading to accumulation of heme precurors
Porphyrias
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
TRAP (tartrate resistant acid phosphatase
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

20. Which immunoglobulin is involved in warm agglutination?
Fibrinogen
Lysine for glutamate at position 6
IgG - Warm is GREAT
CNS and testis

21. What are the etiologies of folate def
Kids= exposure to lead paint - adults = battery - ammunition factory
CD15 and CD30 pos
Tissue factor converst the VII and VIIa
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

22. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Hereditary spherocytosis
Porphobilinogen - delta ALA - uroporphyrin
Hodgkin
Pyruvate kinase def - extravascular

23. What is the coombs test results in AIHA?
Inc serum iron - normal TIBC - inc ferratin
Pos
Dec plasma volume
Blistering cutaneous photosens - most common porphyria

24. Ringed sideroblasts
Blod - tissue - MACS
Sideroblastic anemia
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Polycythemia vera

25. What is the effect of ACE on bradykinin
Sickle cell anemia
Ewing sarcoma
Inactivates it
TPA - cleavage of fibrin mesh

26. Which pathway and factorrs are tested by the PT coag test
Hereditary spherocytosis - autoimmune hemolysis
Helminth infections major basic protein
HbSS
Extrinsic - I - II - V - VII and X

27. What activates the fibrinolytic pathway?
Multiple myeloma
Lysine for glutamate at position 6
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

28. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Aplastic anemia - pancytopenia
Hereditary spherocytosis - G6PD - sickle cell
Ticlopidine/clopidogrel
Signs and sx of aplastic anemia

29. What condition can result from treating AML M3 from the release of the Auer rods
Anemia of chronic disease - Aplastic anemia - kidney disease
Up - down - up
DIC
Hereditary elloptocytosis

30. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Heinz bodies - seen in alpha thal and G6PD
No lytic bone lesions in WM
Round densly staining nucleus with a small amount of pale cytoplasm
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

31. What percentage of WBCs are eosinophils?
1 to 6
Liver disease - abetalipoproteinemia - acntho = spiny
ADP and Ca
Orotic aciduria

32. What is the pathogenesis of TTP?
DIC
Inc large vWF multimers - inc platelet aggregation and thrombosis
5- FU - AZT - hydroxyurea
Helminth infections major basic protein

33. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Epoxide reductase - warfarin inhibits
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
M3 AML (acute promyelocytic leukemia)
Spleen

34. poikilocytosis
Varying shapes
Parvovirus
Low O2 in papilla; can also get microhematuria from medullary infarcts
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

35. inc LDH - jaundice
Extravascular hemolysis
Ringed sideronblasts with iron laden mitochondria
Mantle cell - older males
Up - down - nl

36. Back pain - hemoglobinuria
Common a few days after oxidative stress in a pt with G6PD
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Alpha thal - asian and african american
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

37. What do platelets release necessary for coagulation cascaed?
Leukemia
ADP and Ca
Protoporphyrin (blood)
Uroporphyrinogen decarboxylase

38. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Essential thrombocytosis
EBV
Fe def - thal - pb poison - sideroblastic anemia
VWD

39. Drug that inhibits COX and therefore TXA2 synthesis
Lysine for glutamate at position 6
40 to 75
Howell Jolly bodies
Aspirin

40. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
B12 def
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Blistering cutaneous photosens - most common porphyria
DDAVP (desmopressin) which releases stored vWF stored in endothelium

41. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Factor V resistant to activated protein C's inhibition
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Anti Rh
Inc HbA2 on electrophoresis

42. What is the tx for sickle cell
Sideroblastic anemia
Hydroxyurea - bone marrow transplant
IFN gama
8-10 days

43. What is the life span of a normal RBC
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
120 days
Intrinsic - all factors except - VII - XIII

44. What do you see a starry sky appearance in Burkitt
Spleen
Multiple myeloma
Sheets of lymphocytes interspersed with macs
Low in CML

45. What causes the jaundice in extravascular hemolysis
Langerhans cell histiocytosis
Inc UCB
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
All trans retinoic acid

46. What causes hydrops fetalis
Adult T cell lymphoma - presents with cutaneous lesions
Idiopathic thrombocytopenia (ITP)
Low O2 in papilla; can also get microhematuria from medullary infarcts
Hb Barts - gamma4 in defect in all 4 alpha genes

47. What is the tx for aplastic anemia
Inc RBC - dec O2 sat - inc EPO
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
No lytic bone lesions in WM
Kids= exposure to lead paint - adults = battery - ammunition factory

48. What are the presenting symptoms of acute intermittent porphyria
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 -
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Blistering cutaneous photosens - most common porphyria
5- FU - AZT - hydroxyurea

49. What is hemophilia B?
Megakaryocytes
Histaminase and arylsulfatase
Valine for glutamate
Def in factor IX

50. What is the main source of energy in RBCs
90% anearobically from glucose to lactate - 10% from HMP shunt
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Hereditary spherocytosis
Paraprotein spike - monoclonal protein