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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 the alpha granules contain in platelets?
Lead - and EtOH
Protein S - cleaves and invactivates Va and VIIIa
VWF and fibrinogen
Burkitt lymphoma

2. What is the age breakdown for hodgkins
Essential thrombocytosis
Bimodal - men>women - except for nodular sclerosing type
DDAVP (desmopressin) which releases stored vWF stored in endothelium
CML to AML or All

3. What does LEAD stand for in lead poisoning?
Adult T cell lymphoma - presents with cutaneous lesions
Inc UCB
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

4. Bite cell
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Birbeck granules
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
G6PD

5. Where does All spread
Exposed collagen upon endothelial damage
CNS and testis
Langerhans cell histiocytosis
No lytic bone lesions in WM

6. What is the difference of presentation of Burkitt in Africa vs the United States
Down - up - down
Hereditary spherocytosis
B cells
Africa = Jaw lesion - US = pelvis or abdomen

7. What is their role?
Sickle cell
Inappropriate absolute with inc RBCs and EPO
VIII
Inducers of primary antibody response

8. What virus is associated with 50% of Hodgkin lymphoma
Hereditary elloptocytosis
20 to 40
EBV
Tissue factor converst the VII and VIIa

9. Serum iron - transferrin - ferritin lab values for hemochromatosis
Lung disease - congenital heart diseaes - and high altitude
Up - down - up
Mantle cell - older males
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

10. What vitamin is a cofactor for the first step of heme synthesis
Hereditary spherocytosis - autoimmune hemolysis
B6
Ringed sideronblasts with iron laden mitochondria
B12/folate def

11. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Follicular lymphoma - indolent course
Degrades fibrin mesh and converts C3 to C3a
Abciximab
CLL (SLL without the peripheral lymphocytosis

12. Why can newborns with sickle cell be asymptomatic
Inc RBC - dec O2 sat - inc EPO
Plasma cell
Inc HbF and dec HbS
Valine for glutamate

13. What is the defect in beta thal?
Varying shapes
Splicing sites and promotor sequences
Schistocytes - helmet cells
Orotic aciduria

14. What substance prevents mast cells degranulation?
Intravascular
Cromolyn sodium
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Inducers of primary antibody response

15. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
All trans retinoic acid
B12 def
Multiple myeloma
Lead - and EtOH

16. What is the pathogenesis of sickle cell
Hereditary spherocytosis - autoimmune hemolysis
Varying sizes
DIC
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

17. What are the presenting symptoms of acute intermittent porphyria
Lead - and EtOH
Intrinsic - all factors except - VII - XIII
Bite cells and Heinz bodies
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

18. What is the treatment for sideroblastic anemia
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Blasts > 5%
B6 therapy (pyrodixine)
Inc

19. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Follicular lymphoma - indolent course
SLE - CLL - alpha methyldopa
Examples of oxidative stress
Intravascular

20. Is HS extravascular or intravascular?
HbSS
Fibrinogen bind GpIIb/IIIa and links platelts
Extravascular
Intravascular hemolysis

21. What begins the extrinsic pathway?
Glucose and heme - which inhibit ALA synthase
All trans retinoic acid
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Tissue factor converst the VII and VIIa

22. normal RBCs added to patient's serum
Indirect coombs - agglutinate if serum anti RBC surface Ig
Anti Rh
Schistocytes and inc LDH
Orotic aciduria

23. What virus is associated with Burkitt lymphoma
EBV
Inappropriate absolute with inc RBCs and EPO
Von Willebrand's disease
Plasma cell

24. When do you see MAHA?
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Easy gas exchange
DIC
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

25. Adults present with cutaneous patches/nodules - indolent CD4+
T(12;21)
Mycosis fundgoides/Sezary syndrome
Howell - Jolly bodies - hypo/asplenia
Paroxysmal nocturnal hemoglobinuria

26. Is G6PD intravascular or extravascular
Intravascular
Paroxysmal nocturnal hemoglobinuria
VWF carries/protects factor VIII
Uroporphyrin (tea colored urine)

27. What role does antithrombin play?
Incactivates II - VII - IX - X - XI - XII
They bind vWF via GpIb
CNS and testis
Complications of sickle cell anemia

28. What is the presenting scenario for TTP?
ATIII def
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Lacunar in nodular sclerosing variant

29. What activates protein C and What does activated protein C do?
Inc lymphoctes - and less RS cells
Protein S - cleaves and invactivates Va and VIIIa
Dec synthesis of factors 1972 - protein C/S
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

30. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Alpha thal - asian and african american
Aspirin
Sickle cell anemia
Plasma cell

31. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Porphyrias
No lytic bone lesions in WM
Prothrombin gene mutation

32. What is the receptor for vWF
Leukemia
Imatinib - anti bcr abl
Ewing sarcoma
Gp1b

33. How are the nucleus and the cytoplasm characterized for lymphocytes
Round densly staining nucleus with a small amount of pale cytoplasm
B12 in fatty acid pathways leads to subacute combined degeneration
Inc UCB
Factor V Leiden

34. What state is commonly associated with nonHod lymphoma
Langerhans cells
Ferrocheletase and ALA dehydrogenase
HIV or immunosupression
Malaria - Babesia

35. What are the main associations with multiple myeloma?
Multiple myeloma
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Cromolyn sodium
AML

36. Who are the professional APCs?
Dendritic cells?
Bimodal - men>women - except for nodular sclerosing type
PGI2 - NO inc blood flow - dec platelet aggregation
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

37. Basophilica stippling
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Sickle cell anemia
HIV or immunosupression
Lead - and EtOH

38. What does plasmin do?
Lysosomes
Down - up - down
Degrades fibrin mesh and converts C3 to C3a
M3 AML (acute promyelocytic leukemia)

39. What is the enzyme defect in Porphyria cutanea tarda
Dec
Uroporphyrinogen decarboxylase
Reed - sternberg cells
Lymphoma

40. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Extravascular
Lymphoma
Factor V Leiden

41. Fava beans - sufla drugs - infectinos
Low O2 in papilla; can also get microhematuria from medullary infarcts
Examples of oxidative stress
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Splicing sites and promotor sequences

42. What is the pathogenesis of ACD?
B symptoms - fever night sweats - weight loss
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

43. Which substrates begin the heme synthesis pathway
No antigen - both antibodies
Hereditary spherocytosis - autoimmune hemolysis
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Glycine and succinyl - coa

44. what proteins can be defective in HS?
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Ankryin - band 4.1 or spectrin
Bernard soulier
2 to 10

45. Basophilic nuclear remnants fonud in RBCs
...
CLL (SLL without the peripheral lymphocytosis
Howell - Jolly bodies - hypo/asplenia
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

46. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Leukemoid reaction
Megakaryocytes
MHC II and Fc receptors
Polycythemia vera

47. Fibrotic obliteration of bone marow with teardrop cells

48. drug that inhibits ADP induced expression of GpIIb/IIIa
Ticlopidine/clopidogrel
ADP and Ca
Bernard soulier
Inc HbF and dec HbS

49. What is the accumulated substance in lead poisoning
Protoporphyrin (blood)
Petechiae
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Lead - and EtOH

50. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Ticlopidine/clopidogrel
Alpha thal - asian and african american
Orotic aciduria
Signs and sx of aplastic anemia