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Test your basic knowledge |
Hemeonc
Start Test
Study First
Subject
:
health-sciences
Instructions:
Answer 50 questions in 15 minutes.
If you are not ready to take this test, you can
study here
.
Match each statement with the correct term.
Don't refresh. All questions and answers are randomly picked and ordered every time you load a test.
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 treatment for sideroblastic anemia
Ticlopidine/clopidogrel
B6 therapy (pyrodixine)
Porphobilinogen deaminase aka uroporphyrinogen I synthase
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
2. Bone pain plus anemia
Inappropriate absolute with inc RBCs and EPO
Multiple myeloma
No lytic bone lesions in WM
GpIIb/IIIa
3. What is the enzyme defect in Porphyria cutanea tarda
Inc HbF and dec HbS
Uroporphyrinogen decarboxylase
Langerhans cell histiocytosis
Bimodal - men>women - except for nodular sclerosing type
4. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Factor V Leiden
Aplastic anemia - pancytopenia
Inappropriate absolute with inc RBCs and EPO
SLE - CLL - alpha methyldopa
5. drug that inhibits ADP induced expression of GpIIb/IIIa
Decrease EPO
Plummer - vinson syndrome
Ticlopidine/clopidogrel
No lytic bone lesions in WM
6. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Thrombotic thrombocytopenic purpura
Intravascular hemolysis
Her next Rh+ fetus
Bernard soulier
7. hypocellular bone marrown tih fatty infiltration
Valine for glutamate
Aplastic anemia - pancytopenia
Ferrocheletase and ALA dehydrogenase
B6 therapy (pyrodixine)
8. What signal activates MACS
Fe def - thal - pb poison - sideroblastic anemia
IFN gama
GpIIb/IIIa
Ticlopidine/clopidogrel
9. Who are the professional APCs?
B12/folate def
Dendritic cells?
Polycythemia vera
Valine for glutamate
10. 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
Sickle cell
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Ferrocheletase and ALA dehydrogenase
Lysosomes
11. What cell is primarily involved in non Hod lymph
Epoxide reductase - warfarin inhibits
HbSS
Peroxidase
B cells
12. What does 'Neutrophils Like Making Everything Better' stand for?
Mantle cell lymphoma
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
13. What is the affected enzyme in lead poisoning
Inc RBC - dec O2 sat - inc EPO
Fe def - thal - pb poison - sideroblastic anemia
Ferrocheletase and ALA dehydrogenase
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
14. How does vWD cause elevated PTT?
Examples of oxidative stress
No antigen - both antibodies
Imatinib - anti bcr abl
VWF carries/protects factor VIII
15. iron deficiency anemia - esophageal web - atrophic glossitis
Ferrocheletase and ALA dehydrogenase
Plummer - vinson syndrome
Complications of sickle cell anemia
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
16. What is makes a leukemia acute
Imatinib - anti bcr abl
B6
2 to 10
Blasts > 5%
17. What virus can cause an aplastic crisis in pts with HS?
Parvovirus
Yes - via MHC II
T(9;22) bcr abl
No lytic bone lesions in WM
18. Which drugs can cause macrocytic anemia?
40 to 75
5- FU - AZT - hydroxyurea
Inc HbA2 on electrophoresis
Acts to stabilize platelet plug
19. Adults present with cutaneous patches/nodules - indolent CD4+
Low O2 in papilla; can also get microhematuria from medullary infarcts
Mycosis fundgoides/Sezary syndrome
Beta chain underproduced - asymptomatic - heterozygote
DDAVP (desmopressin) which releases stored vWF stored in endothelium
20. Drug that inhbits the GpIIb/IIIa directly
Ankryin - band 4.1 or spectrin
Plummer - vinson syndrome
Abciximab
Nodular sclerosing
21. What does STOP Making New Thrombi stand for
B12 def
Pos
Histamine - heparin - and eosinohil chemotactic factors
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
22. t(11:22)
Low O2 in papilla; can also get microhematuria from medullary infarcts
Multiple myeloma
Ewing sarcoma
VIII
23. What causes the physiologic chloride shift and What does the chloride shift do?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Protein S - cleaves and invactivates Va and VIIIa
Adult T cell lymphoma - presents with cutaneous lesions
40 to 75
24. X linked - dec glutathione inc RBC susceptibility to oxidative stress
G6PD
A antigen and B antibodies
Tissue
All
25. What activates the fibrinolytic pathway?
All trans retinoic acid
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Sickle cell anemia
Bone marrow - thymus - blood (80% of circulating lymphos are T)
26. What are the variants of the RS cells
Ferrocheletase and ALA dehydrogenase
Burkitt lymphoma
Lacunar in nodular sclerosing variant
Lead - and EtOH
27. Ddx for microcytic anemia
Signs and sx of aplastic anemia
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Fe def - thal - pb poison - sideroblastic anemia
Up - down - up
28. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Hereditary spherocytosis
Incactivates II - VII - IX - X - XI - XII
Indirect coombs - agglutinate if serum anti RBC surface Ig
Bleeding - malnutrition/absorption - inc demand pregs
29. RBCs damagaed from passing through obstructed - narrowed vessel lumina
120 days
B12/folate def
MAHA
B6 therapy (pyrodixine)
30. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
VWD
DIC
Lysine for glutamate at position 6
B6
31. Conditions of defective heme synthesis leading to accumulation of heme precurors
CNS and testis
Prothrombin gene mutation
Porphyrias
Common a few days after oxidative stress in a pt with G6PD
32. What are the pro aggregation factors?
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Incactivates II - VII - IX - X - XI - XII
TXA2 - dec blood flow - inc platelet aggregation
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
33. What is the effect of ACE on bradykinin
Anti Rh
Inactivates it
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Paraprotein spike - monoclonal protein
34. What is hemophiliia A
MGUS - monoclonal gammopathy of undetermined significance
Deficiency in factor VIII
Blod - tissue - MACS
Nl - up - nl
35. Serum iron - transferrin - ferritin lab values for pregs - OCP use
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Nl - up - nl
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Intravascular hemolysis
36. What does increasing heme do to ALA synthase activity
GpIIb/IIIa
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Dec
Sideroblastic anemia
37. What does Vit K deficiency cause?
T(9;22) bcr abl
Dec synthesis of factors 1972 - protein C/S
Hereditary spherocytosis - G6PD - sickle cell
Lung disease - congenital heart diseaes - and high altitude
38. What is the presenting scenario for TTP?
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
IFN gama
Blistering cutaneous photosens - most common porphyria
Langerhans cells
39. What is the age group most commonly affected by multiple myeloma
Burkitt lymphoma
40 to 50
CML
Bite cells and Heinz bodies
40. What are some causes of cold agglutinin anemia
B12/folate def
Low O2 in papilla; can also get microhematuria from medullary infarcts
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Liver disease - abetalipoproteinemia - acntho = spiny
41. What does antithrombin do and What activates it?
Lacunar in nodular sclerosing variant
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Produce antibody - lots of RER and golgi
No
42. Mutation in 3' untranslated region associated with venous clots
AML
Orotic aciduria
ATIII def
Prothrombin gene mutation
43. What is the therapy for the M3 variant?
Hereditary spherocytosis
All trans retinoic acid
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Paroxysmal nocturnal hemoglobinuria
44. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
Varying sizes
B12 def
Dec
45. What is the affected enzyme in acute intermittment porphyria
Porphobilinogen - delta ALA - uroporphyrin
Histamine - heparin - and eosinohil chemotactic factors
TXA2 - dec blood flow - inc platelet aggregation
CD15 and CD30 pos
46. What condition can result from treating AML M3 from the release of the Auer rods
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
G6PD
DIC
Extravascular hemolysis
47. What is a metabolic disorder tht can cause macrocytic anemia?
Polycythemia vera
Epoxide reductase - warfarin inhibits
Valine for glutamate
Orotic aciduria
48. Does CML have a JAK2 mut
No
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
SLE - CLL - alpha methyldopa
Inc serum iron - normal TIBC - inc ferratin
49. What is the Ddx for a normocytic - normochromic anemia?
Antigen - antibody
Extrinsic - I - II - V - VII and X
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Hereditary spherocytosis
50. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
SLE - CLL - alpha methyldopa
Sickle cell
No lytic bone lesions in WM
TRAP (tartrate resistant acid phosphatase