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Hemeonc

Instructions:

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. Acanthocyte (spur cell)
Inc UCB
Blood
Liver disease - abetalipoproteinemia - acntho = spiny
No lytic bone lesions in WM

2. What is the ddx for aplastic anemia

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3. t(11:22)
B12/folate def
Ewing sarcoma
Yes - via MHC II
Degrades fibrin mesh and converts C3 to C3a

4. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
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 -
Folate def
Myelofibrosis (marow is crying being its fibrosed'
Howell - Jolly bodies - hypo/asplenia

5. Who are the professional APCs?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Dendritic cells?
Blod - tissue - MACS
Extrinsic - I - II - V - VII and X

6. What does antithrombin do and What activates it?
Fibrinogen bind GpIIb/IIIa and links platelts
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Lead - and EtOH
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

7. What percentage of WBCs are eosinophils?
1 to 6
Sideroblastic anemia
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Aplastic anemia - pancytopenia

8. What do the platelets bind? What is the step called
Extrinsic - I - II - V - VII and X
Protoporphyrin (blood)
Contiguous
They bind vWF via GpIb

9. What does bradykinin do?
Inc vasodiltion - inc perm - inc pain
Blood
Cromolyn sodium
Leukemia

10. Adults present with cutaneous patches/nodules - indolent CD4+
Nl - up - nl
Idiopathic thrombocytopenia (ITP)
Complications of sickle cell anemia
Mycosis fundgoides/Sezary syndrome

11. What virus is associated with 50% of Hodgkin lymphoma
Uroporphyrin (tea colored urine)
Megakaryocytes
Porphyrias
EBV

12. Target cell

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13. Deficiency in vWF

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14. What is the characteristic finding for MAHA on peripheral smear?
Schistocytes - helmet cells
Extravascular
IgG - Warm is GREAT
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

15. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Sickle cell anemia
Intravascular hemolysis
Multiple myeloma
Extravascular hemolysis

16. What is the main source of energy in RBCs
DIC - TTP/HUS - traumatic hemolysis
90% anearobically from glucose to lactate - 10% from HMP shunt
Blistering cutaneous photosens - most common porphyria
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

17. What is the tx for sickle cell
Hydroxyurea - bone marrow transplant
Porphobilinogen - delta ALA - uroporphyrin
B cells
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

18. What does LEAD stand for in lead poisoning?
Inc large vWF multimers - inc platelet aggregation and thrombosis
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
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 -

19. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
Examples of oxidative stress
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
No lytic bone lesions in WM

20. inc HbF on electrophoresis
Both alpha and beta thal
Lymphoma
Dendritic cells?
Protoporphyrin (blood)

21. what proteins can be defective in HS?
Uroporphyrinogen decarboxylase
Inc lymphoctes - and less RS cells
Heinz bodies - seen in alpha thal and G6PD
Ankryin - band 4.1 or spectrin

22. What is the treatment for sideroblastic anemia
DDAVP (desmopressin) which releases stored vWF stored in endothelium
40 to 75
B6 therapy (pyrodixine)
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

23. What reveresible things can a sideroblastic anemia
Lead - and EtOH
Up - down - nl
Paraprotein spike - monoclonal protein
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

24. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Burkitt lymphoma
Inappropriate absolute with inc RBCs and EPO
Bleeding - malnutrition/absorption - inc demand pregs

25. discrete tumor masses arising from lymph nodes
SLE - CLL - alpha methyldopa
Inc RBC - dec O2 sat - inc EPO
Lymphoma
Heinz bodies - seen in alpha thal and G6PD

26. Bite cell
G6PD
TRAP (tartrate resistant acid phosphatase
Megakaryocytes
Low in CML

27. What is a metabolic disorder tht can cause macrocytic anemia?
Intravascular
Orotic aciduria
Mantle cell lymphoma
Collagen - BM - activated platelets

28. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Schistocytes and inc LDH
CLL (SLL without the peripheral lymphocytosis
Sideroblastic anemia
Incactivates II - VII - IX - X - XI - XII

29. What is the age breakdown for hodgkins
Protoporphyrin (blood)
Ringed sideronblasts with iron laden mitochondria
Bimodal - men>women - except for nodular sclerosing type
MHC II and Fc receptors

30. What is the most common hodgkin lymphoma
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Nodular sclerosing
8-10 days
Glycine and succinyl - coa

31. What substance prevents mast cells degranulation?
T(9;22) bcr abl
Blistering cutaneous photosens - most common porphyria
Plasma cell
Cromolyn sodium

32. What is factor V leidin?

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33. Where does All spread
Sideroblastic anemia
CNS and testis
Incactivates II - VII - IX - X - XI - XII
Prothrombin gene mutation

34. What is the Ddx for nonhemolytic normocytic anemia
Anemia of chronic disease - Aplastic anemia - kidney disease
Varying sizes
Extravascular
Lysosomes

35. What is the presenting scenario for TTP?
G6PD
Uroporphyrinogen decarboxylase
Abciximab
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

36. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Her next Rh+ fetus
Porphobilinogen - delta ALA - uroporphyrin
Protoporphyrin
40 to 50

37. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Tissue factor converst the VII and VIIa
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Down - down - up
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 -

38. Where are mast cells found
Tissue
Hereditary elloptocytosis
Orotic aciduria
B symptoms - fever night sweats - weight loss

39. What does 'Neutrophils Like Making Everything Better' stand for?
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Helps platelts adhere to endothelium
Antigen - antibody
Both alpha and beta thal

40. What activates the intrinsic pathway?
Myelofibrosis (marow is crying being its fibrosed'
MGUS - monoclonal gammopathy of undetermined significance
B12 def
Collagen - BM - activated platelets

41. What activates the fibrinolytic pathway?
Inactivates it
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
90% anearobically from glucose to lactate - 10% from HMP shunt
MAHA

42. How are plasma cells characterized?
Folate def
ATIII def
Off center nuclues - clock face chromatin
Plummer - vinson syndrome

43. In hemophilia A or B What do you see in the coag tests
Nl PT - elevated PTT - intrinsic pathway defect
All
120 days
Decrease EPO

44. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Howell Jolly bodies
Blod - tissue - MACS
CLL (SLL without the peripheral lymphocytosis
Intravascular

45. What is the tx for vWD
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Ticlopidine/clopidogrel
Varying sizes

46. Which cell is neoplastic in multiple myeloma
Glycine and succinyl - coa
Plasma cell
Spleen
B12/folate def

47. What do you see on peripheral smear with sideroblastic anemia
Ringed sideronblasts with iron laden mitochondria
Up - down - nl
Factor V Leiden
Paroxysmal nocturnal hemoglobinuria

48. Elliptocyte
Inc UCB
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Hereditary elloptocytosis

49. What do plasma cells do
AML
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Produce antibody - lots of RER and golgi
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

50. anisocytosis
Easy gas exchange
AML
Hereditary spherocytosis - autoimmune hemolysis
Varying sizes