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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. Megakaryocytosis
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Protein S - cleaves and invactivates Va and VIIIa
Essential thrombocytosis
Birbeck granules

2. What are the variants of the RS cells
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Lacunar in nodular sclerosing variant
Protoporphyrin (blood)
Ticlopidine/clopidogrel

3. What is the defect in beta thal?
Splicing sites and promotor sequences
Langerhans cells
Varying shapes
B symptoms - fever night sweats - weight loss

4. How are the nucleus and the cytoplasm characterized for lymphocytes
Induces differentiation of myeloblasts
Beta 4
Round densly staining nucleus with a small amount of pale cytoplasm
Inc HbF and dec HbS

5. What is the accumulated substance in lead poisoning
Spleen
Liver disease - abetalipoproteinemia - acntho = spiny
Protoporphyrin (blood)
Leukemia

6. What is the life spance of a platelet?
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Beta 4
8-10 days
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

7. What causes hydrops fetalis
Hb Barts - gamma4 in defect in all 4 alpha genes
VIII
Hodgkin
5- FU - AZT - hydroxyurea

8. What are the etiologies of folate def
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Thrombotic thrombocytopenic purpura
Intrinsic - all factors except - VII - XIII

9. What is the tx for aplastic anemia
Parvovirus
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Burkitt lymphoma
120 days

10. What happens in beta thal major?
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
B antigena and A antibodes
T(12;21)

11. What finding you do you see in patients after splenectomy
Howell Jolly bodies
VWF and fibrinogen
Glucose and heme - which inhibit ALA synthase
Alpha thal - asian and african american

12. Which immunoglobulin is involved in warm agglutination?
IgG - Warm is GREAT
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Mycosis fundgoides/Sezary syndrome
TXA2 - dec blood flow - inc platelet aggregation

13. What is the affected enzyme in lead poisoning
Ferrocheletase and ALA dehydrogenase
Inc HbF and dec HbS
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

14. What is the mutation in HbC
Lysine for glutamate at position 6
Fibrinogen
Nodular sclerosing
DDAVP (desmopressin) which releases stored vWF stored in endothelium

15. What do the iron studies show in sideroblastic anemia
Inc serum iron - normal TIBC - inc ferratin
Protein C or S deficiency
Hereditary spherocytosis - G6PD - sickle cell
Inc UCB

16. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
CML to AML or All
Beta 4
Leukemia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

17. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Inc
Birbeck granules

18. What state is commonly associated with nonHod lymphoma
Inc UCB
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
HIV or immunosupression
Nl - up - nl

19. What is appropriate absolute polycythemia
Inc RBC - dec O2 sat - inc EPO
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Def in factor IX
Down - down - up

20. What is the receptor for fibrinogen?
B12 def
GpIIb/IIIa
Contiguous
Burkitt lymphoma

21. Who are the professional APCs?
Dendritic cells?
They bind vWF via GpIb
Histamine - heparin - and eosinohil chemotactic factors
VWD

22. Serum iron - transferrin - ferritin lab values for pregs - OCP use
EBV
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Nl - up - nl
Protein S - cleaves and invactivates Va and VIIIa

23. What are the four levels of alpha thal?
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
G6PD
Pyruvate kinase def - extravascular
Malaria - Babesia

24. What is the pattern of involvement and spread for nonHod lympho
Sickle cell anemia
Valine for glutamate
Glucose and heme - which inhibit ALA synthase
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

25. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Uroporphyrin (tea colored urine)
Anti Rh
Hereditary spherocytosis - autoimmune hemolysis
Inducers of primary antibody response

26. What virus is associated with 50% of Hodgkin lymphoma
EBV
All trans retinoic acid
T(12;21)
Down - up - down

27. Elderly - mature b cell tumor with filamentous - hairlike projections
Hairy cell leukemia
DIC
Bite cells and Heinz bodies
M3 AML (acute promyelocytic leukemia)

28. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
Thrombotic thrombocytopenic purpura
M3 AML (acute promyelocytic leukemia)
Inactivates it
Sickle cell

29. What is the ddx for aplastic anemia

30. What signal activates MACS
Lacunar in nodular sclerosing variant
Polycythemia vera
IFN gama
Inducers of primary antibody response

31. What do you see a starry sky appearance in Burkitt
Sheets of lymphocytes interspersed with macs
20 to 40
Direct coombs - agglutinate if RBCs are coated with Ig
Howell Jolly bodies

32. What activates the intrinsic pathway?
Signs and sx of aplastic anemia
Collagen - BM - activated platelets
Nl - up - nl
Ferrocheletase and ALA dehydrogenase

33. What is the pathogenesis of ACD?
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
...
VWF and fibrinogen
Extravascular

34. Which substrates begin the heme synthesis pathway
Glycine and succinyl - coa
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Protoporphyrin
B6 therapy (pyrodixine)

35. What is the tx for vWD
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Multiple myeloma
HbSS
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

36. What cell is primarily involved in non Hod lymph
B cells
Thrombotic thrombocytopenic purpura
Schistocytes - helmet cells
Nl PT - elevated PTT - intrinsic pathway defect

37. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
B symptoms - fever night sweats - weight loss
MHC II and Fc receptors
Folate def

38. What are the anti aggregation factors?
Dec synthesis of factors 1972 - protein C/S
Def in factor IX
PGI2 - NO inc blood flow - dec platelet aggregation
Sickle cell

39. What is the treatment for lead poising?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
2 to 10
Sickle cell anemia

40. What is the purpose of the fibrin mesh
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Sideroblastic anemia
Acts to stabilize platelet plug

41. defect in alpha globin gene - dec alpha globin synth - disease and populations?
DIC
Anemia of chronic disease - Aplastic anemia - kidney disease
Alpha thal - asian and african american
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

42. What substance is Fe added to to yield heme
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Inactivates it
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Protoporphyrin

43. inc HbF on electrophoresis
Both alpha and beta thal
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Antigen - antibody
Megakaryocytes

44. What percentage if WBCs are polys?
Examples of oxidative stress
40 to 75
EBV
TXA2 - dec blood flow - inc platelet aggregation

45. Bite cell
Howell - Jolly bodies - hypo/asplenia
Essential thrombocytosis
G6PD
B6

46. normal RBCs added to patient's serum
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Indirect coombs - agglutinate if serum anti RBC surface Ig
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

47. Where to T cells arise and mature - and migrate to...
VIII
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Exposed collagen upon endothelial damage
B antigena and A antibodes

48. From what cells are platelets derived from
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Bimodal - men>women - except for nodular sclerosing type
Megakaryocytes
Rhogam - Rh antigen immunoglobulin

49. When is the peak incidence for nonHod lymphoma
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
CML
20 to 40
GpIIb/IIIa

50. What percentage of WBCs are basophils - and What is found in their basophilic granules?
Protein S - cleaves and invactivates Va and VIIIa
Deficiency in factor VIII
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
No platelet clumping