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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. What is their role?
HIV or immunosupression
Bite cells and Heinz bodies
Mantle cell - older males
Inducers of primary antibody response

2. What is a metabolic disorder tht can cause macrocytic anemia?
Orotic aciduria
Hereditary spherocytosis - autoimmune hemolysis
Lysine for glutamate at position 6
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

3. What is the therapy for CML (philly chrom)
B12 in fatty acid pathways leads to subacute combined degeneration
120 days
T(9;22) bcr abl
Imatinib - anti bcr abl

4. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
No antigen - both antibodies
Extravascular
Factor V Leiden

5. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
DIC
Diffuse Large b cell lymphoma
Indirect coombs - agglutinate if serum anti RBC surface Ig
No

6. Fava beans - sufla drugs - infectinos
Examples of oxidative stress
Orotic aciduria
Porphobilinogen - delta ALA - uroporphyrin
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

7. S-100 and CD1a with birbeck granules
Intravascular
Deficiency in factor VIII
Pyruvate kinase def - extravascular
Langerhans cell histiocytosis

8. What virus is associated with 50% of Hodgkin lymphoma
Acts to stabilize platelet plug
Direct coombs - agglutinate if RBCs are coated with Ig
EBV
Exposed collagen upon endothelial damage

9. defect in alpha globin gene - dec alpha globin synth - disease and populations?
ADP and Ca
Protein S - cleaves and invactivates Va and VIIIa
Paroxysmal nocturnal hemoglobinuria
Alpha thal - asian and african american

10. Which cell is neoplastic in multiple myeloma
Nodular sclerosing
Anemia of chronic disease - Aplastic anemia - kidney disease
120 days
Plasma cell

11. What are the presenting symptoms of acute intermittent porphyria
Porphobilinogen - delta ALA - uroporphyrin
Alpha thal - asian and african american
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Inappropriate absolute with inc RBCs and EPO

12. What is the pathogenesis of TTP?
Contiguous
DIC
Blistering cutaneous photosens - most common porphyria
Inc large vWF multimers - inc platelet aggregation and thrombosis

13. What is monoclonal expansion without symptoms associated with multiple myeloma?
HbSS
Produce antibody - lots of RER and golgi
MGUS - monoclonal gammopathy of undetermined significance
Heinz bodies - seen in alpha thal and G6PD

14. What do you see in vit K def
Hereditary elloptocytosis
Howell Jolly bodies
Birbeck granules
Eleveated PT - PTT

15. What is the characteristic spread of Hodgkin Lymphoma
Idiopathic thrombocytopenia (ITP)
Sideroblastic anemia
Hodgkin
Contiguous

16. Ddx for microcytic anemia
Fe def - thal - pb poison - sideroblastic anemia
T(12;21)
Bleeding - malnutrition/absorption - inc demand pregs
DDAVP (desmopressin) which releases stored vWF stored in endothelium

17. What does decreasing heme do to ALA synthase activity?
Anti Rh
Intravascular hemolysis
Inc
Porphyrias

18. What is appropriate absolyte polycythemia associated with
Round densly staining nucleus with a small amount of pale cytoplasm
GpIIb/IIIa
Lung disease - congenital heart diseaes - and high altitude
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

19. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Leukemoid reaction
Lead - and EtOH
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Multiple myeloma

20. From what cells are platelets derived from
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Megakaryocytes
CD15 and CD30 pos
ADP and Ca

21. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Fe def - thal - pb poison - sideroblastic anemia
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
G6PD
Idiopathic thrombocytopenia (ITP)

22. What is the life span of a normal RBC
VWD
120 days
Ferrocheletase and ALA dehydrogenase
Uroporphyrinogen decarboxylase

23. CD5+ - poor prognosis - t(11;14)
Sickle cell anemia
Mantle cell - older males
Hydroxyurea - bone marrow transplant
Thrombotic thrombocytopenic purpura

24. inc HbF on electrophoresis
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Multiple myeloma
Both alpha and beta thal
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

25. What do auer rods stain with
Incactivates II - VII - IX - X - XI - XII
Peroxidase
AB - no antibodies
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

26. Defect in platelet to platelet adhesion - receptor - disorder - platelet count and bleeding time

27. What causes hydrops fetalis
Nl - up - nl
Hb Barts - gamma4 in defect in all 4 alpha genes
Gp1b
Aspirin

28. What causes the jaundice in extravascular hemolysis
Inc UCB
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Epoxide reductase - warfarin inhibits
Varying shapes

29. What does bradykinin do?
Mycosis fundgoides/Sezary syndrome
Abciximab
Histamine - heparin - and eosinohil chemotactic factors
Inc vasodiltion - inc perm - inc pain

30. What is the affected enzyme in acute intermittment porphyria
Myelofibrosis (marow is crying being its fibrosed'
They bind vWF via GpIb
Porphobilinogen - delta ALA - uroporphyrin
Dec

31. What is the characteristic finding for MAHA on peripheral smear?
Sheets of lymphocytes interspersed with macs
120 days
Schistocytes - helmet cells
Protein C or S deficiency

32. Upregulated growth of leukocytes in bone marro
Paroxysmal nocturnal hemoglobinuria
Sheets of lymphocytes interspersed with macs
Fc
Leukemia

33. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Inc lymphoctes - and less RS cells
Spleen
Paroxysmal nocturnal hemoglobinuria

34. What is the tx for sickle cell
Hydroxyurea - bone marrow transplant
PGI2 - NO inc blood flow - dec platelet aggregation
A antigen and B antibodies
Complications of sickle cell anemia

35. What is the affected enzyme in lead poisoning
Glanzmann's throbmasthenia
Bone marrow - thymus - blood (80% of circulating lymphos are T)
T(9;22) bcr abl
Ferrocheletase and ALA dehydrogenase

36. What is the pathogenesis of aplastic anemia with kidney disease
Produce antibody - lots of RER and golgi
Intravascular
Decrease EPO
VWD

37. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
CLL (SLL without the peripheral lymphocytosis
Birbeck granules
Degrades fibrin mesh and converts C3 to C3a
Lacunar in nodular sclerosing variant

38. Target cell

39. What are the variants of the RS cells
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
EBV
Lacunar in nodular sclerosing variant
Complications of sickle cell anemia

40. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Inducers of primary antibody response
Burkitt lymphoma
Intrinsic - all factors except - VII - XIII
G6PD

41. What is the receptor for fibrinogen?
GpIIb/IIIa
Schistocytes and inc LDH
Prothrombin gene mutation
All

42. What is the age group most commonly affected by multiple myeloma
Liver disease - abetalipoproteinemia - acntho = spiny
40 to 50
Mantle cell lymphoma
Dendritic cells?

43. Does CML have a JAK2 mut
Plummer - vinson syndrome
Splicing sites and promotor sequences
Uroporphyrinogen decarboxylase
No

44. What do platelets interact with to form a hemostatic plug
Liver disease - abetalipoproteinemia - acntho = spiny
Schistocytes - helmet cells
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Fibrinogen

45. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Heinz bodies - seen in alpha thal and G6PD
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Complications of sickle cell anemia
IFN gama

46. Why does B12 def cause neuro sx?
B12 in fatty acid pathways leads to subacute combined degeneration
IgG - Warm is GREAT
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 -
Protein C or S deficiency

47. What substance prevents mast cells degranulation?
Cromolyn sodium
IFN gama
Dec
Megakaryocytes

48. What are the etiologies of B12 def
IFN gama
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

49. HTLV-1
All
Adult T cell lymphoma - presents with cutaneous lesions
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

50. What do you see on peripheral smear with sideroblastic anemia
Ringed sideronblasts with iron laden mitochondria
Reed - sternberg cells
Plasma cell
Incactivates II - VII - IX - X - XI - XII