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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 the accumulated substance in lead poisoning
Inducers of primary antibody response
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Decrease EPO
Protoporphyrin (blood)

2. CD5+ - poor prognosis - t(11;14)
Langerhans cells
Mantle cell - older males
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
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 -

3. hypocellular bone marrown tih fatty infiltration
DIC
Aplastic anemia - pancytopenia
Abciximab
Hereditary spherocytosis - G6PD - sickle cell

4. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Folate def
Lacunar in nodular sclerosing variant
40 to 75
VWF carries/protects factor VIII

5. What is the pathogenesis of aplastic anemia with kidney disease
Decrease EPO
PGI2 - NO inc blood flow - dec platelet aggregation
Inc
Inc lymphoctes - and less RS cells

6. What is the pathogenesis of sickle cell
Fc
TXA2 - dec blood flow - inc platelet aggregation
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Folate def

7. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
Bone marrow infiltration - myelofibrosis
GpIIb/IIIa
Howell Jolly bodies
No lytic bone lesions in WM

8. What does the blood smear show in glanzmann's?
No platelet clumping
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Bone marrow infiltration - myelofibrosis
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

9. What is the most common nonhodgkin lymphoma
Folate def
Ewing sarcoma
Diffuse Large b cell lymphoma
Lysosomes

10. Can B cells function as APCs?
Yes - via MHC II
Inc large vWF multimers - inc platelet aggregation and thrombosis
Glanzmann's throbmasthenia
Antigen - antibody

11. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Pos
Hodgkin
Aspirin
B6

12. What do platelets interact with to form a hemostatic plug
Dec plasma volume
No platelet clumping
Fibrinogen
Alpha thal - asian and african american

13. What does the large SA:volume ratio in RBCs help facilitate?
Easy gas exchange
VWF carries/protects factor VIII
Histamine - heparin - and eosinohil chemotactic factors
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

14. What is the philadelphia chromosome
Macrohemorrhage - hemarthroses - easy bruising
Off center nuclues - clock face chromatin
T(9;22) bcr abl
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

15. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Helps platelts adhere to endothelium
Hereditary elloptocytosis
Heinz bodies - seen in alpha thal and G6PD
CD15 and CD30 pos

16. What substance prevents mast cells degranulation?
All trans retinoic acid
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
No platelet clumping
Cromolyn sodium

17. What do you see on peripheral smear with sideroblastic anemia
Yes - via MHC II
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Ringed sideronblasts with iron laden mitochondria
20 to 40

18. universal donor
Peroxidase
PGI2 - NO inc blood flow - dec platelet aggregation
No antigen - both antibodies
Bite cells and Heinz bodies

19. What causes hydrops fetalis
Hb Barts - gamma4 in defect in all 4 alpha genes
EBV
Leukemoid reaction
Hodgkin

20. poikilocytosis
40 to 50
Anemia of chronic disease - Aplastic anemia - kidney disease
Inc HbA2 on electrophoresis
Varying shapes

21. What is appropriate absolyte polycythemia associated with
Aspirin
Lung disease - congenital heart diseaes - and high altitude
Produce antibody - lots of RER and golgi
VWF and fibrinogen

22. What CD molecules are on RS cells
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
CD15 and CD30 pos
DIC
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

23. What are the presenting symptoms of porphyria cutanea tarda
Lung disease - congenital heart diseaes - and high altitude
Blistering cutaneous photosens - most common porphyria
Essential thrombocytosis
ADP and Ca

24. What does increasing heme do to ALA synthase activity
Inappropriate absolute with inc RBCs and EPO
Dec
Liver disease - abetalipoproteinemia - acntho = spiny
Porphyrias

25. What does Vit K deficiency cause?
Hydroxyurea - bone marrow transplant
Leukemia
Dec synthesis of factors 1972 - protein C/S
Intrinsic - all factors except - VII - XIII

26. What is the therapy for CML (philly chrom)
TRAP (tartrate resistant acid phosphatase
Imatinib - anti bcr abl
Inc lymphoctes - and less RS cells
Eleveated PT - PTT

27. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
B12/folate def
No

28. Bite cell
Uroporphyrinogen decarboxylase
Common a few days after oxidative stress in a pt with G6PD
Hydroxyurea - bone marrow transplant
G6PD

29. hemolytic in a newborn - dec ATP and rigid RBCs
Pyruvate kinase def - extravascular
Acts to stabilize platelet plug
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Signs and sx of aplastic anemia

30. t(15;17)
M3 AML (acute promyelocytic leukemia)
120 days
Fibrinogen bind GpIIb/IIIa and links platelts
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

31. Where does All spread
Bimodal - men>women - except for nodular sclerosing type
Gp1b
CNS and testis
Up - down - nl

32. normal RBCs added to patient's serum
Hairy cell leukemia
Indirect coombs - agglutinate if serum anti RBC surface Ig
Low O2 in papilla; can also get microhematuria from medullary infarcts
Multiple myeloma

33. What do the labs show for TTP?
Schistocytes and inc LDH
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

34. What is the pattern of involvement and spread for nonHod lympho
HbSS
Antigen - antibody
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Lymphoma

35. What is the age group most commonly affected by multiple myeloma
40 to 50
DIC
Ankryin - band 4.1 or spectrin
Leukemia

36. What is hemophiliia A
Plummer - vinson syndrome
AB - no antibodies
Deficiency in factor VIII
40 to 75

37. What cell is primarily involved in non Hod lymph
Burkitt lymphoma
20 to 40
Blasts > 5%
B cells

38. Ddx for microcytic anemia
TXA2 - dec blood flow - inc platelet aggregation
VWD
Inc HbF and dec HbS
Fe def - thal - pb poison - sideroblastic anemia

39. What is the age breakdown for hodgkins
Examples of oxidative stress
Bimodal - men>women - except for nodular sclerosing type
Dec synthesis of factors 1972 - protein C/S
Inc vasodiltion - inc perm - inc pain

40. What does hairy cell leukemia stain with
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
G6PD
TRAP (tartrate resistant acid phosphatase
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

41. What percentage if WBCs are polys?
Examples of oxidative stress
Imatinib - anti bcr abl
Follicular lymphoma - indolent course
40 to 75

42. What is relative polycythemia
Dec plasma volume
Varying shapes
Indirect coombs - agglutinate if serum anti RBC surface Ig
SLE - CLL - alpha methyldopa

43. tennis rackets on EM
Howell Jolly bodies
Inactivates it
No platelet clumping
Birbeck granules

44. Which immunoglobulin is involved in warm agglutination?
Essential thrombocytosis
Africa = Jaw lesion - US = pelvis or abdomen
Hydroxyurea - bone marrow transplant
IgG - Warm is GREAT

45. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
TXA2 - dec blood flow - inc platelet aggregation
Intravascular
No lytic bone lesions in WM
Histaminase and arylsulfatase

46. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Beta thal
Epoxide reductase - warfarin inhibits
Inc RBC - dec O2 sat - inc EPO
40 to 50

47. Why does B12 def cause neuro sx?
CNS and testis
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
B12 in fatty acid pathways leads to subacute combined degeneration
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

48. What is the characteristic histologic finding in Hodgkin Lymphoma
Varying shapes
Reed - sternberg cells
G6PD
B12 def

49. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Anti Rh
Protein C or S deficiency
120 days

50. What is the most common hodgkin lymphoma
Inc
SLE - CLL - alpha methyldopa
Plasma cell
Nodular sclerosing