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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. What is the pattern of involvement and spread for nonHod lympho
AML
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

2. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Factor V Leiden
Pyruvate kinase def - extravascular

3. How does vWD cause elevated PTT?
Hairy cell leukemia
VWF carries/protects factor VIII
Sickle cell anemia
Acts to stabilize platelet plug

4. What are the anti aggregation factors?
PGI2 - NO inc blood flow - dec platelet aggregation
Porphobilinogen - delta ALA - uroporphyrin
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Splicing sites and promotor sequences

5. What is contained within the azurophilic granules of PMNs
Hb Barts - gamma4 in defect in all 4 alpha genes
Easy gas exchange
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
VWD

6. What do auer rods stain with
No platelet clumping
Megakaryocytes
Peroxidase
Extravascular

7. What is their role?
40 to 50
Schistocytes - helmet cells
Inducers of primary antibody response
5- FU - AZT - hydroxyurea

8. What percentage if WBCs are polys?
Multiple myeloma
IFN gama
B12 def
40 to 75

9. inc LDH - jaundice
40 to 75
Uroporphyrinogen decarboxylase
Extravascular hemolysis
5- FU - AZT - hydroxyurea

10. Fibrotic obliteration of bone marow with teardrop cells

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11. What are the variants of the RS cells
Mantle cell - older males
DDAVP (desmopressin) which releases stored vWF stored in endothelium
B12/folate def
Lacunar in nodular sclerosing variant

12. What do labs show in DIC?
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Tissue
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
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 -

13. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
CD15 and CD30 pos
Hodgkin
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Leukemia

14. Which pathway and factors are tested in the PTT test
Intrinsic - all factors except - VII - XIII
Antigen - antibody
Aplastic anemia - pancytopenia
Birbeck granules

15. adults - auer rods - inc circulating myeblasts on peripheral smear
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
AML
Exposed collagen upon endothelial damage
Deficiency in factor VIII

16. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Leukemia
Intravascular hemolysis
They bind vWF via GpIb
Alpha thal - asian and african american

17. Where do B cells arise from - mature - and migrate to...
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
HbSS
Produce antibody - lots of RER and golgi
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

18. When do you see MAHA?
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Mantle cell - older males
Glanzmann's throbmasthenia

19. What does 'Neutrophils Like Making Everything Better' stand for?
B antigena and A antibodes
VWF and fibrinogen
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Inc serum iron - normal TIBC - inc ferratin

20. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Nl - up - nl
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Plasma cell
No platelet clumping

21. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Decrease EPO
Inappropriate absolute with inc RBCs and EPO
Intravascular hemolysis

22. Which cell is neoplastic in multiple myeloma
VWD
Plasma cell
Fibrinogen
Sideroblastic anemia

23. What symptoms are associated with Hodgkin lymphoma
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
VIII
Histamine - heparin - and eosinohil chemotactic factors
B symptoms - fever night sweats - weight loss

24. What activates protein C and What does activated protein C do?
Thrombotic thrombocytopenic purpura
Protein S - cleaves and invactivates Va and VIIIa
Degrades fibrin mesh and converts C3 to C3a
120 days

25. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Her next Rh+ fetus
Megakaryocytes
Hereditary spherocytosis - autoimmune hemolysis

26. What is the pathogenesis of aplastic anemia with kidney disease
Incactivates II - VII - IX - X - XI - XII
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Decrease EPO
Collagen - BM - activated platelets

27. What is the difference of presentation of Burkitt in Africa vs the United States
Dec synthesis of factors 1972 - protein C/S
Gp1b
Birbeck granules
Africa = Jaw lesion - US = pelvis or abdomen

28. What do platelets release necessary for coagulation cascaed?
Induces differentiation of myeloblasts
Inc vasodiltion - inc perm - inc pain
Diffuse Large b cell lymphoma
ADP and Ca

29. Acanthocyte (spur cell)
Inactivates it
VWD
Hodgkin
Liver disease - abetalipoproteinemia - acntho = spiny

30. What is the therapy for CML (philly chrom)
Extravascular
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Inc HbA2 on electrophoresis
Imatinib - anti bcr abl

31. What are some causes of warm agglutinin autoimmune hemolytic anemia?
CD15 and CD30 pos
SLE - CLL - alpha methyldopa
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

32. 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
Protoporphyrin
120 days
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Sickle cell

33. What does ectopic EPO produce
MGUS - monoclonal gammopathy of undetermined significance
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Inappropriate absolute with inc RBCs and EPO
Glucose and heme - which inhibit ALA synthase

34. mother's antibodies attack fetal RBCs
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Diffuse Large b cell lymphoma
Inc serum iron - normal TIBC - inc ferratin
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

35. What is the general pathology of a macrocytic anemia?
Ringed sideronblasts with iron laden mitochondria
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Tissue factor converst the VII and VIIa
Inappropriate absolute with inc RBCs and EPO

36. Blood type A
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
CD15 and CD30 pos
A antigen and B antibodies
Inactivates it

37. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Histaminase and arylsulfatase
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
G6PD

38. What are the presenting symptoms of acute intermittent porphyria
Down - down - up
AB - no antibodies
Bimodal - men>women - except for nodular sclerosing type
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

39. What causes the physiologic chloride shift and What does the chloride shift do?
Dec
Spleen
DIC
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

40. What is the characteristic spread of Hodgkin Lymphoma
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
HIV or immunosupression
Contiguous
AB - no antibodies

41. What is the treatment for acute intermittent porphyria
Glycine and succinyl - coa
Glucose and heme - which inhibit ALA synthase
Paroxysmal nocturnal hemoglobinuria
Acts to stabilize platelet plug

42. Deficiency in GpIIb/IIIa

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43. Schisotcyte - helmet cell
MGUS - monoclonal gammopathy of undetermined significance
DIC - TTP/HUS - traumatic hemolysis
Both alpha and beta thal
Low in CML

44. What finding you do you see in patients after splenectomy
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Howell Jolly bodies
Splicing sites and promotor sequences
VIII

45. Serum iron - transferrin - ferritin lab values for hemochromatosis
Up - down - nl
DIC - TTP/HUS - traumatic hemolysis
Antigen - antibody
Up - down - up

46. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Anti Rh
EBV
Inc serum iron - normal TIBC - inc ferratin
Glanzmann's throbmasthenia

47. Elliptocyte
20 to 40
Hereditary spherocytosis - G6PD - sickle cell
Splicing sites and promotor sequences
Hereditary elloptocytosis

48. Which factor does vWF carry/protect
Inc UCB
VIII
Mantle cell lymphoma
Beta chain underproduced - asymptomatic - heterozygote

49. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Porphyrias
Polycythemia vera
Diffuse Large b cell lymphoma
Dec synthesis of factors 1972 - protein C/S

50. How is beta thal minor dx
Howell Jolly bodies
Inc HbA2 on electrophoresis
Thrombotic thrombocytopenic purpura
Exposed collagen upon endothelial damage