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1. What begins the extrinsic pathway?
Tissue factor converst the VII and VIIa
Ferrocheletase and ALA dehydrogenase
Fibrinogen
Diffuse Large b cell lymphoma

2. What do you see in vit K def
T(12;21)
Porphobilinogen - delta ALA - uroporphyrin
Eleveated PT - PTT
IgG - Warm is GREAT

3. How are plasma cells characterized?
Off center nuclues - clock face chromatin
AB - no antibodies
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Eleveated PT - PTT

4. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Diffuse Large b cell lymphoma
Nl - up - nl
Histamine - heparin - and eosinohil chemotactic factors
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 -

5. What are the four levels of alpha thal?
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Glanzmann's throbmasthenia
TXA2 - dec blood flow - inc platelet aggregation

6. How is beta thal minor dx
Lysine for glutamate at position 6
Fibrinogen bind GpIIb/IIIa and links platelts
Inc HbA2 on electrophoresis
B cells

7. Elliptocyte
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Hereditary elloptocytosis
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

8. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Langerhans cells
Essential thrombocytosis
Leukemia
1 to 6

9. What is the general pathology of a macrocytic anemia?
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
VIII
Paraprotein spike - monoclonal protein

10. What is the affected enzyme in lead poisoning
Schistocytes - helmet cells
B12 def
Inc lymphoctes - and less RS cells
Ferrocheletase and ALA dehydrogenase

11. Ringed sideroblasts
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Inc serum iron - normal TIBC - inc ferratin
Langerhans cells
Sideroblastic anemia

12. What are the neuro sx of B12 def?
IgG - Warm is GREAT
Ferrocheletase and ALA dehydrogenase
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
IFN gama

13. What state is commonly associated with nonHod lymphoma
ADP and Ca
T(12;21)
Mycosis fundgoides/Sezary syndrome
HIV or immunosupression

14. Which pathway and factors are tested in the PTT test
Sideroblastic anemia
Howell - Jolly bodies - hypo/asplenia
Intrinsic - all factors except - VII - XIII
Hb Barts - gamma4 in defect in all 4 alpha genes

15. anisocytosis
Varying sizes
40 to 75
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Tissue

16. What is the enzyme defect in Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
1 to 6
B12/folate def
Inc UCB

17. What chromosomal translocation is associated with a better prognosis in All
Paraprotein spike - monoclonal protein
T(12;21)
Abciximab
Diffuse Large b cell lymphoma

18. What is appropriate absolyte polycythemia associated with
Sideroblastic anemia
Lung disease - congenital heart diseaes - and high altitude
Alpha thal - asian and african american
Ankryin - band 4.1 or spectrin

19. Where do B cells arise from - mature - and migrate to...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
TXA2 - dec blood flow - inc platelet aggregation
Factor V Leiden
90% anearobically from glucose to lactate - 10% from HMP shunt

20. Where does All spread
CNS and testis
Schistocytes and inc LDH
Dec synthesis of factors 1972 - protein C/S
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

21. What is the characteristic lab finding on electrophoresis
Extrinsic - I - II - V - VII and X
Paraprotein spike - monoclonal protein
Lymphoma
Nodular sclerosing

22. What is the accumulated substance in lead poisoning
Inc RBC - dec O2 sat - inc EPO
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Hereditary elloptocytosis
Protoporphyrin (blood)

23. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Cromolyn sodium
CLL (SLL without the peripheral lymphocytosis
DIC
Fibrinogen bind GpIIb/IIIa and links platelts

24. Mutation in 3' untranslated region associated with venous clots
Inc RBC - dec O2 sat - inc EPO
Off center nuclues - clock face chromatin
Examples of oxidative stress
Prothrombin gene mutation

25. What substance prevents mast cells degranulation?
Cromolyn sodium
Inducers of primary antibody response
Intravascular
No lytic bone lesions in WM

26. What is the mutation in HbS
Idiopathic thrombocytopenia (ITP)
Valine for glutamate
Myelofibrosis (marow is crying being its fibrosed'
Examples of oxidative stress

27. What causes the jaundice in extravascular hemolysis
Inc UCB
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
VWF and fibrinogen
Blasts > 5%

28. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Intravascular
Nl PT - elevated PTT - intrinsic pathway defect
Imatinib - anti bcr abl
Hodgkin

29. What does Vit K deficiency cause?
IFN gama
Bite cells and Heinz bodies
Dec synthesis of factors 1972 - protein C/S
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

30. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
Pos
They bind vWF via GpIb
Inc HbA2 on electrophoresis
All

31. What is the receptor for fibrinogen?
VWF carries/protects factor VIII
GpIIb/IIIa
Lysine for glutamate at position 6
Inc vasodiltion - inc perm - inc pain

32. What happens in beta thal major?
Common a few days after oxidative stress in a pt with G6PD
Hairy cell leukemia
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
TRAP (tartrate resistant acid phosphatase

33. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Leukemoid reaction
A antigen and B antibodies
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Heinz bodies - seen in alpha thal and G6PD

34. What is the result of thrombocytopenia or platelet dysfunction?
Nodular sclerosing
Folate def
Petechiae
Protoporphyrin (blood)

35. what proteins can be defective in HS?
Inc RBC - dec O2 sat - inc EPO
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Ankryin - band 4.1 or spectrin
40 to 50

36. What do platelets release necessary for coagulation cascaed?
ADP and Ca
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Prothrombin gene mutation
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

37. What is the most common nonhodgkin lymphoma
Helps platelts adhere to endothelium
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Fc
Diffuse Large b cell lymphoma

38. What do labs show in ITP?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
HIV or immunosupression

39. What does 'Neutrophils Like Making Everything Better' stand for?
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Mantle cell - older males
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

40. Upregulated growth of leukocytes in bone marro
Leukemoid reaction
Low in CML
Leukemia
Pyruvate kinase def - extravascular

41. What do you see in peripheral smear in a pt with G6PD?
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
90% anearobically from glucose to lactate - 10% from HMP shunt
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Bite cells and Heinz bodies

42. What virus is associated with Burkitt lymphoma
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 -
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
EBV

43. What is appropriate absolute polycythemia
Inc RBC - dec O2 sat - inc EPO
Ewing sarcoma
Langerhans cells
Contiguous

44. Which substrates begin the heme synthesis pathway
Glycine and succinyl - coa
TPA - cleavage of fibrin mesh
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Produce antibody - lots of RER and golgi

45. What is the effect of ACE on bradykinin
Inactivates it
8-10 days
Lysosomes
5- FU - AZT - hydroxyurea

46. What are the etiologies of folate def
DIC
Heinz bodies - seen in alpha thal and G6PD
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Mycosis fundgoides/Sezary syndrome

47. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
T(12;21)
Lacunar in nodular sclerosing variant
Up - down - nl
Inc UCB

48. What is the pathogenesis of aplastic anemia with kidney disease
Decrease EPO
B12 def
Signs and sx of aplastic anemia
Sideroblastic anemia

49. What activates the intrinsic pathway?
Sickle cell
Spleen
No
Collagen - BM - activated platelets

50. What does LEAD stand for in lead poisoning?
Factor V Leiden
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 -
Down - down - up
Gp1b

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