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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. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Folate def
Bone marrow infiltration - myelofibrosis
Mantle cell lymphoma
Paroxysmal nocturnal hemoglobinuria

2. What is the pattern of involvement and spread for nonHod lympho
Megakaryocytes
Varying sizes
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Induces differentiation of myeloblasts

3. Serum iron - transferrin - ferritin lab values for iron def anemia
Protoporphyrin (blood)
Down - up - down
Round densly staining nucleus with a small amount of pale cytoplasm
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

4. Bone pain plus anemia
Eleveated PT - PTT
Multiple myeloma
2 to 10
20 to 40

5. What is their role?
Eleveated PT - PTT
Inducers of primary antibody response
Direct coombs - agglutinate if RBCs are coated with Ig
Imatinib - anti bcr abl

6. What condition can result from treating AML M3 from the release of the Auer rods
M3 AML (acute promyelocytic leukemia)
MGUS - monoclonal gammopathy of undetermined significance
Ewing sarcoma
DIC

7. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
IFN gama
Hodgkin
Folate def
Pos

8. What indicated a good prognosis in Hodgkin lymphoma?
Direct coombs - agglutinate if RBCs are coated with Ig
Inc lymphoctes - and less RS cells
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Abciximab

9. What reveresible things can a sideroblastic anemia
Fe def - thal - pb poison - sideroblastic anemia
Histaminase and arylsulfatase
Lead - and EtOH
Glucose and heme - which inhibit ALA synthase

10. t(15;17)
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Bone marrow infiltration - myelofibrosis
M3 AML (acute promyelocytic leukemia)
Her next Rh+ fetus

11. Fava beans - sufla drugs - infectinos
Yes - via MHC II
Examples of oxidative stress
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

12. What causes the jaundice in extravascular hemolysis
Schistocytes - helmet cells
Sideroblastic anemia
Inc UCB
Complications of sickle cell anemia

13. Drug that inhibits COX and therefore TXA2 synthesis
Sideroblastic anemia
Aspirin
A antigen and B antibodies
Follicular lymphoma - indolent course

14. HTLV-1
5- FU - AZT - hydroxyurea
Adult T cell lymphoma - presents with cutaneous lesions
Intravascular
Up - down - up

15. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Lead - and EtOH
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Hereditary spherocytosis
Up - down - up

16. What chromosomal translocation is associated with a better prognosis in All
T(12;21)
T(9;22) bcr abl
Sheets of lymphocytes interspersed with macs
Porphobilinogen - delta ALA - uroporphyrin

17. What is HbH
Direct coombs - agglutinate if RBCs are coated with Ig
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Beta 4
8-10 days

18. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Low in CML
T(12;21)
Protein S - cleaves and invactivates Va and VIIIa

19. What does hairy cell leukemia stain with
TRAP (tartrate resistant acid phosphatase
Rhogam - Rh antigen immunoglobulin
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

20. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Common a few days after oxidative stress in a pt with G6PD
Acts to stabilize platelet plug
TXA2 - dec blood flow - inc platelet aggregation
Epoxide reductase - warfarin inhibits

21. What is hemophiliia A
ADP and Ca
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Deficiency in factor VIII
5- FU - AZT - hydroxyurea

22. philadelphia chromosome - blood looks like marrow
Essential thrombocytosis
Birbeck granules
CML
Up - down - nl

23. What is the most common nonhodgkin lymphoma
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Aspirin
Anemia of chronic disease - Aplastic anemia - kidney disease
Diffuse Large b cell lymphoma

24. What are the presenting symptoms of acute intermittent porphyria
Hereditary spherocytosis - G6PD - sickle cell
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Bernard soulier
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

25. What is appropriate absolute polycythemia
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
B cells
Inc RBC - dec O2 sat - inc EPO
Positive osmootic fragility test and splenectomy

26. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
AML
Polycythemia vera
DIC
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

27. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Protein S - cleaves and invactivates Va and VIIIa
Examples of oxidative stress
G6PD
B12/folate def

28. What do you see a starry sky appearance in Burkitt
Multiple myeloma
No antigen - both antibodies
Sheets of lymphocytes interspersed with macs
Direct coombs - agglutinate if RBCs are coated with Ig

29. What are the etiologies of folate def
Beta chain underproduced - asymptomatic - heterozygote
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
GpIIb/IIIa

30. What is the tx for vWD
Direct coombs - agglutinate if RBCs are coated with Ig
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Sideroblastic anemia
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

31. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Decrease EPO
SLE - CLL - alpha methyldopa
Ferrocheletase and ALA dehydrogenase

32. What does increasing heme do to ALA synthase activity
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Dec
8-10 days
CD15 and CD30 pos

33. CD5+ - poor prognosis - t(11;14)
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Mantle cell - older males
Down - up - down
Inc UCB

34. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Hereditary spherocytosis - G6PD - sickle cell
Her next Rh+ fetus

35. poikilocytosis
Varying shapes
5- FU - AZT - hydroxyurea
Anti Rh
B12 def

36. What are the labs and tx for HS?
Glanzmann's throbmasthenia
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
B antigena and A antibodes
Positive osmootic fragility test and splenectomy

37. Which drugs can cause macrocytic anemia?
Africa = Jaw lesion - US = pelvis or abdomen
5- FU - AZT - hydroxyurea
Inappropriate absolute with inc RBCs and EPO
Bleeding - malnutrition/absorption - inc demand pregs

38. RBCs damagaed from passing through obstructed - narrowed vessel lumina
MAHA
Paraprotein spike - monoclonal protein
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Bone marrow infiltration - myelofibrosis

39. Basophilic nuclear remnants fonud in RBCs
Howell - Jolly bodies - hypo/asplenia
Inc large vWF multimers - inc platelet aggregation and thrombosis
ATIII def
B6 therapy (pyrodixine)

40. What is makes a leukemia acute
Blasts > 5%
Histaminase and arylsulfatase
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 -
Eleveated PT - PTT

41. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
B12 in fatty acid pathways leads to subacute combined degeneration
Leukemia
Intravascular hemolysis
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

42. What are the anti aggregation factors?
Paraprotein spike - monoclonal protein
Macrohemorrhage - hemarthroses - easy bruising
VIII
PGI2 - NO inc blood flow - dec platelet aggregation

43. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Indirect coombs - agglutinate if serum anti RBC surface Ig
Incactivates II - VII - IX - X - XI - XII
Easy gas exchange
VWD

44. What do eosinophils defend against and What do they use to do it?
8-10 days
Idiopathic thrombocytopenia (ITP)
Helminth infections major basic protein
Acts to stabilize platelet plug

45. t(11:22)
HbSS
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Ewing sarcoma
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

46. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Anti Rh
Down - down - up
SLE - CLL - alpha methyldopa
No antigen - both antibodies

47. What is the activation stage of platelet plug formation?
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Factor V resistant to activated protein C's inhibition
Varying shapes
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

48. What finding you do you see in patients after splenectomy
Howell Jolly bodies
B12 in fatty acid pathways leads to subacute combined degeneration
IgG - Warm is GREAT
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

49. What is hemophilia B?
Def in factor IX
HbSS
Gp1b
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

50. What is the result of thrombocytopenia or platelet dysfunction?
Petechiae
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Von Willebrand's disease
Beta thal