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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. Where are basophils found?
Blood
Both alpha and beta thal
Mantle cell - older males
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

2. RBCs damagaed from passing through obstructed - narrowed vessel lumina
MAHA
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Aplastic anemia - pancytopenia
AB - no antibodies

3. What do plasma cells do
Common a few days after oxidative stress in a pt with G6PD
Produce antibody - lots of RER and golgi
Blod - tissue - MACS
Essential thrombocytosis

4. What substance accumulates in porphyria cutanea
Nodular sclerosing
5- FU - AZT - hydroxyurea
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Uroporphyrin (tea colored urine)

5. S-100 and CD1a with birbeck granules
MGUS - monoclonal gammopathy of undetermined significance
MHC II and Fc receptors
Langerhans cell histiocytosis
CML

6. t(11;14)
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Multiple myeloma
Incactivates II - VII - IX - X - XI - XII
Mantle cell lymphoma

7. What is the age group most commonly affected by multiple myeloma
Malaria - Babesia
ATIII def
Indirect coombs - agglutinate if serum anti RBC surface Ig
40 to 50

8. What is the treatment for sideroblastic anemia
B12 in fatty acid pathways leads to subacute combined degeneration
Hodgkin
Up - down - up
B6 therapy (pyrodixine)

9. What indicated a good prognosis in Hodgkin lymphoma?
Sickle cell anemia
Inc lymphoctes - and less RS cells
Low O2 in papilla; can also get microhematuria from medullary infarcts
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

10. What is the effected enzyme in acute intermittment porphyria?
ADP and Ca
Multiple myeloma
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Porphobilinogen deaminase aka uroporphyrinogen I synthase

11. What do you see in peripheral smear in a pt with G6PD?
Down - up - down
PGI2 - NO inc blood flow - dec platelet aggregation
Bite cells and Heinz bodies
Bone marrow - thymus - blood (80% of circulating lymphos are T)

12. Sickle cell
Sickle cell anemia
Ankryin - band 4.1 or spectrin
Essential thrombocytosis
Inc serum iron - normal TIBC - inc ferratin

13. What is the enzyme defect in Porphyria cutanea tarda
DIC - TTP/HUS - traumatic hemolysis
Folate def
Epoxide reductase - warfarin inhibits
Uroporphyrinogen decarboxylase

14. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
Megakaryocytes
Lead - and EtOH
Degrades fibrin mesh and converts C3 to C3a
No lytic bone lesions in WM

15. What is the main source of energy in RBCs
VWF and fibrinogen
Lysine for glutamate at position 6
GpIIb/IIIa
90% anearobically from glucose to lactate - 10% from HMP shunt

16. Who are the professional APCs?
Dendritic cells?
Langerhans cell histiocytosis
Hereditary spherocytosis - G6PD - sickle cell
Bite cells and Heinz bodies

17. What do platelets interact with to form a hemostatic plug
20 to 40
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Fibrinogen
Leukemia

18. What is the coombs test results in AIHA?
Epoxide reductase - warfarin inhibits
Pos
Paraprotein spike - monoclonal protein
2 to 10

19. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Burkitt lymphoma
Hereditary spherocytosis
They bind vWF via GpIb
Decrease EPO

20. anisocytosis
Signs and sx of aplastic anemia
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Inc
Varying sizes

21. After an injury - What does vWF bind to begin platelet plug formation?
CLL (SLL without the peripheral lymphocytosis
Blistering cutaneous photosens - most common porphyria
Exposed collagen upon endothelial damage
Inducers of primary antibody response

22. What is the characteristic histologic finding in Hodgkin Lymphoma
MHC II and Fc receptors
Reed - sternberg cells
Bone marrow infiltration - myelofibrosis
Easy gas exchange

23. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
M3 AML (acute promyelocytic leukemia)
Lung disease - congenital heart diseaes - and high altitude
Idiopathic thrombocytopenia (ITP)
Inc UCB

24. What is the affected enzyme in acute intermittment porphyria
CML to AML or All
Dendritic cells?
DIC
Porphobilinogen - delta ALA - uroporphyrin

25. What is the result of thrombocytopenia or platelet dysfunction?
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
CNS and testis
Fibrinogen
Petechiae

26. Basophilica stippling
Nodular sclerosing
TXA2 - dec blood flow - inc platelet aggregation
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Blasts > 5%

27. What do they express on their surface?
Nodular sclerosing
MHC II and Fc receptors
Plasma cell
Easy gas exchange

28. Fibrotic obliteration of bone marow with teardrop cells

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29. Mutation in 3' untranslated region associated with venous clots
Folate def
Prothrombin gene mutation
Sideroblastic anemia
Bone marrow - thymus - blood (80% of circulating lymphos are T)

30. What are some causes of cold agglutinin anemia
Ticlopidine/clopidogrel
Extravascular
Her next Rh+ fetus
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

31. what proteins can be defective in HS?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Lung disease - congenital heart diseaes - and high altitude
Ankryin - band 4.1 or spectrin
MAHA

32. Conditions of defective heme synthesis leading to accumulation of heme precurors
Porphyrias
G6PD
Thrombotic thrombocytopenic purpura
Pyruvate kinase def - extravascular

33. What is a metabolic disorder tht can cause macrocytic anemia?
Pyruvate kinase def - extravascular
Low O2 in papilla; can also get microhematuria from medullary infarcts
Orotic aciduria
Round densly staining nucleus with a small amount of pale cytoplasm

34. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Beta 4
Low in CML
SLE - CLL - alpha methyldopa
A antigen and B antibodies

35. Who has more severe disease - HbSS or HbSC
Extravascular
Common a few days after oxidative stress in a pt with G6PD
HbSS
Fibrinogen

36. Blood type B
1 to 6
B antigena and A antibodes
Dec
Easy gas exchange

37. iron deficiency anemia - esophageal web - atrophic glossitis
G6PD
Helminth infections major basic protein
Hereditary spherocytosis
Plummer - vinson syndrome

38. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
They bind vWF via GpIb
Lead - and EtOH
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
B12 def

39. Which infections can cause MAHA?
Helminth infections major basic protein
Ticlopidine/clopidogrel
Malaria - Babesia
Porphobilinogen deaminase aka uroporphyrinogen I synthase

40. What is their role?
VWF carries/protects factor VIII
Nl - up - nl
Inducers of primary antibody response
All

41. What is the clinical picture of hemophilia A or B
Easy gas exchange
Inappropriate absolute with inc RBCs and EPO
Macrohemorrhage - hemarthroses - easy bruising
EBV

42. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Low in CML
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Uroporphyrinogen decarboxylase
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

43. What is the defect in beta thal?
Abciximab
Paroxysmal nocturnal hemoglobinuria
Splicing sites and promotor sequences
ADP and Ca

44. t(8;14) c - myc gene
Factor V resistant to activated protein C's inhibition
Extrinsic - I - II - V - VII and X
Hydroxyurea - bone marrow transplant
Burkitt lymphoma

45. Plasma cell neoplasm
Multiple myeloma
Down - down - up
G6PD
CML

46. What cell is primarily involved in non Hod lymph
Schistocytes and inc LDH
Uroporphyrinogen decarboxylase
Complications of sickle cell anemia
B cells

47. Which drugs can cause macrocytic anemia?
Inc lymphoctes - and less RS cells
Tissue factor converst the VII and VIIa
5- FU - AZT - hydroxyurea
Spleen

48. What does hairy cell leukemia stain with
Indirect coombs - agglutinate if serum anti RBC surface Ig
B12 in fatty acid pathways leads to subacute combined degeneration
Plasma cell
TRAP (tartrate resistant acid phosphatase

49. What percentage of WBCs are basophils - and What is found in their basophilic granules?
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Inc serum iron - normal TIBC - inc ferratin
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Diffuse Large b cell lymphoma

50. normal RBCs added to patient's serum
Indirect coombs - agglutinate if serum anti RBC surface Ig
Low O2 in papilla; can also get microhematuria from medullary infarcts
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Bone marrow - thymus - blood (80% of circulating lymphos are T)