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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. How does the therapy for M3 vairant work?
T(9;22) bcr abl
Spleen
Induces differentiation of myeloblasts
Hereditary elloptocytosis

2. What is factor V leidin?

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3. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Induces differentiation of myeloblasts
Leukemoid reaction
Blistering cutaneous photosens - most common porphyria
Lead - and EtOH

4. bcl -2 t(14;18) adults
1 to 6
Dec plasma volume
Follicular lymphoma - indolent course
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

5. What are the main associations with multiple myeloma?
Bone marrow infiltration - myelofibrosis
G6PD
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Def in factor IX

6. What is the tx for vWD
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Porphobilinogen deaminase aka uroporphyrinogen I synthase
SLE - CLL - alpha methyldopa
Factor V resistant to activated protein C's inhibition

7. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
Lysosomes
Diffuse Large b cell lymphoma
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

8. What does ADP do?
Inducers of primary antibody response
No antigen - both antibodies
Helps platelts adhere to endothelium
Hairy cell leukemia

9. drug that inhibits ADP induced expression of GpIIb/IIIa
Sickle cell
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Ticlopidine/clopidogrel
Prothrombin gene mutation

10. What are some classic examples of extravascular hemolysis
Idiopathic thrombocytopenia (ITP)
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Hereditary spherocytosis - G6PD - sickle cell
Bimodal - men>women - except for nodular sclerosing type

11. What is the characteristic spread of Hodgkin Lymphoma
Contiguous
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Pos
Helps platelts adhere to endothelium

12. What does antithrombin do and What activates it?
20 to 40
VWF and fibrinogen
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Inappropriate absolute with inc RBCs and EPO

13. Ringed sideroblasts
2 to 10
Sideroblastic anemia
Malaria - Babesia
Inc serum iron - normal TIBC - inc ferratin

14. What are the age ranges for the various leukemias
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Deficiency in factor VIII
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Schistocytes - helmet cells

15. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Heinz bodies - seen in alpha thal and G6PD
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
VWD
40 to 50

16. Which cell is neoplastic in multiple myeloma
Follicular lymphoma - indolent course
Schistocytes and inc LDH
Plasma cell
Inc serum iron - normal TIBC - inc ferratin

17. What state is commonly associated with nonHod lymphoma
Easy gas exchange
8-10 days
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
HIV or immunosupression

18. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Paroxysmal nocturnal hemoglobinuria
Malaria - Babesia
Pyruvate kinase def - extravascular

19. Adults present with cutaneous patches/nodules - indolent CD4+
Protoporphyrin (blood)
Mycosis fundgoides/Sezary syndrome
Malaria - Babesia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

20. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
M3 AML (acute promyelocytic leukemia)
Hereditary spherocytosis
Hereditary elloptocytosis
Sheets of lymphocytes interspersed with macs

21. How are plasma cells characterized?
B cells
Off center nuclues - clock face chromatin
DIC
Gp1b

22. What percentage of WBCs are eosinophils?
Helps platelts adhere to endothelium
Von Willebrand's disease
1 to 6
Varying shapes

23. universal donor
AML
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
No antigen - both antibodies
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

24. What do eosinophils defend against and What do they use to do it?
Signs and sx of aplastic anemia
Varying sizes
Helminth infections major basic protein
Fibrinogen

25. what proteins can be defective in HS?
Aspirin
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Collagen - BM - activated platelets
Ankryin - band 4.1 or spectrin

26. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Beta chain underproduced - asymptomatic - heterozygote
Inc HbF and dec HbS
Protein C or S deficiency
Her next Rh+ fetus

27. What is the therapy for the M3 variant?
Schistocytes - helmet cells
All trans retinoic acid
Extravascular hemolysis
Ticlopidine/clopidogrel

28. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Leukemia
DIC
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Inc serum iron - normal TIBC - inc ferratin

29. universal recipient
AB - no antibodies
120 days
Inc
Lacunar in nodular sclerosing variant

30. What is the general pathology of a macrocytic anemia?
CD15 and CD30 pos
Extravascular hemolysis
Complications of sickle cell anemia
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

31. What are the azuraphilic granules in PMNs
B12 def
Induces differentiation of myeloblasts
ATIII def
Lysosomes

32. What do you see in vit K def
Langerhans cells
Eleveated PT - PTT
Rhogam - Rh antigen immunoglobulin
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

33. anisocytosis
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Langerhans cells
Varying sizes

34. Target cell

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35. What is hemophilia B?
Both alpha and beta thal
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
A antigen and B antibodies
Def in factor IX

36. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
Glucose and heme - which inhibit ALA synthase
Peroxidase
ATIII def
SLE - CLL - alpha methyldopa

37. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
No antigen - both antibodies
Hereditary spherocytosis
Her next Rh+ fetus
Plummer - vinson syndrome

38. What do you see a starry sky appearance in Burkitt
Howell Jolly bodies
Sheets of lymphocytes interspersed with macs
Glycine and succinyl - coa
Uroporphyrin (tea colored urine)

39. What indicated a good prognosis in Hodgkin lymphoma?
Inc lymphoctes - and less RS cells
Beta thal
Schistocytes - helmet cells
Spleen

40. RBCs damagaed from passing through obstructed - narrowed vessel lumina
Aplastic anemia - pancytopenia
Hairy cell leukemia
Megakaryocytes
MAHA

41. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Lead - and EtOH
Dendritic cells?
Up - down - nl
Valine for glutamate

42. hemolytic in a newborn - dec ATP and rigid RBCs
5- FU - AZT - hydroxyurea
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 -
Pyruvate kinase def - extravascular
Hydroxyurea - bone marrow transplant

43. t(8;14) c - myc gene
Burkitt lymphoma
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Megakaryocytes

44. What does the large SA:volume ratio in RBCs help facilitate?
40 to 75
Antigen - antibody
8-10 days
Easy gas exchange

45. What is the mutation in HbS
Valine for glutamate
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Exposed collagen upon endothelial damage
B12 in fatty acid pathways leads to subacute combined degeneration

46. What do you see on peripheral smear with sideroblastic anemia
1 to 6
Dec
Ringed sideronblasts with iron laden mitochondria
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

47. anti - Ig antibody added to patients RBCs;
Malaria - Babesia
Direct coombs - agglutinate if RBCs are coated with Ig
Schistocytes - helmet cells
Aplastic anemia - pancytopenia

48. What are dendritic cells called in the skin?
Lead - and EtOH
Inc vasodiltion - inc perm - inc pain
Langerhans cells
Folate def

49. When do you see MAHA?
Orotic aciduria
Hereditary spherocytosis
Paraprotein spike - monoclonal protein
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

50. What does STOP Making New Thrombi stand for
Ticlopidine/clopidogrel
Sickle cell
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Porphobilinogen deaminase aka uroporphyrinogen I synthase