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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 are the azuraphilic granules in PMNs
Langerhans cell histiocytosis
Collagen - BM - activated platelets
Lysosomes
Positive osmootic fragility test and splenectomy

2. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Contiguous
Porphobilinogen - delta ALA - uroporphyrin
Leukemia
Factor V Leiden

3. What is the characteristic spread of Hodgkin Lymphoma
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Contiguous
Mycosis fundgoides/Sezary syndrome
Lead - and EtOH

4. What is the affected enzyme in acute intermittment porphyria
B12 def
Porphobilinogen - delta ALA - uroporphyrin
Direct coombs - agglutinate if RBCs are coated with Ig
Peroxidase

5. What the alpha granules contain in platelets?
VWF and fibrinogen
Macrohemorrhage - hemarthroses - easy bruising
Inactivates it
SLE - CLL - alpha methyldopa

6. What is their role?
Inducers of primary antibody response
GpIIb/IIIa
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Down - up - down

7. What are dendritic cells called in the skin?
Von Willebrand's disease
Langerhans cells
Inc
Fe def - thal - pb poison - sideroblastic anemia

8. What is the tx for vWD
Prothrombin gene mutation
DDAVP (desmopressin) which releases stored vWF stored in endothelium
EBV
Inc RBC - dec O2 sat - inc EPO

9. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Africa = Jaw lesion - US = pelvis or abdomen
Sideroblastic anemia
Factor V resistant to activated protein C's inhibition
Hodgkin

10. Bone pain plus anemia
B6
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Multiple myeloma
They bind vWF via GpIb

11. What signal activates MACS
IFN gama
Bone marrow infiltration - myelofibrosis
Blood
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

12. What is the pathogenesis of sickle cell
Varying sizes
Fibrinogen bind GpIIb/IIIa and links platelts
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
AML

13. What is hemophiliia A
Contiguous
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Up - down - nl
Deficiency in factor VIII

14. What is the pattern of involvement and spread for nonHod lympho
Plummer - vinson syndrome
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Yes - via MHC II
T(12;21)

15. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Birbeck granules
Dec synthesis of factors 1972 - protein C/S
Nodular sclerosing
SLE - CLL - alpha methyldopa

16. Teardrop cell
Multiple myeloma
Nodular sclerosing
Protoporphyrin
Bone marrow infiltration - myelofibrosis

17. Which drugs can cause macrocytic anemia?
Hairy cell leukemia
Megakaryocytes
Complications of sickle cell anemia
5- FU - AZT - hydroxyurea

18. From what cells are platelets derived from
Positive osmootic fragility test and splenectomy
Mantle cell - older males
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Megakaryocytes

19. What is the most common nonhodgkin lymphoma
G6PD
20 to 40
T(12;21)
Diffuse Large b cell lymphoma

20. What activates the intrinsic pathway?
Collagen - BM - activated platelets
Protein C or S deficiency
Beta thal
Bernard soulier

21. poikilocytosis
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Varying shapes
Hb Barts - gamma4 in defect in all 4 alpha genes
Africa = Jaw lesion - US = pelvis or abdomen

22. What CD molecules are on RS cells
Indirect coombs - agglutinate if serum anti RBC surface Ig
Folate def
Schistocytes and inc LDH
CD15 and CD30 pos

23. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Ringed sideronblasts with iron laden mitochondria
Tissue factor converst the VII and VIIa
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
B12 def

24. What virus is associated with Burkitt lymphoma
Dec synthesis of factors 1972 - protein C/S
EBV
DIC
Beta thal

25. Where are monocytes typically found - where do they go - and What do the differentiate into?
VWF carries/protects factor VIII
Blod - tissue - MACS
EBV
AML

26. What is the characteristic lab finding on electrophoresis
CLL (SLL without the peripheral lymphocytosis
Reed - sternberg cells
G6PD
Paraprotein spike - monoclonal protein

27. What do the iron studies show in sideroblastic anemia
Schistocytes and inc LDH
Blood
VWF carries/protects factor VIII
Inc serum iron - normal TIBC - inc ferratin

28. What does increasing heme do to ALA synthase activity
Dec
Helminth infections major basic protein
Adult T cell lymphoma - presents with cutaneous lesions
Exposed collagen upon endothelial damage

29. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
MAHA
Signs and sx of aplastic anemia
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Protoporphyrin (blood)

30. Megakaryocytosis
Essential thrombocytosis
2 to 10
Cromolyn sodium
Hodgkin

31. What does antithrombin do and What activates it?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Protoporphyrin
Bite cells and Heinz bodies
Dec synthesis of factors 1972 - protein C/S

32. What is the receptor for fibrinogen?
Both alpha and beta thal
GpIIb/IIIa
Multiple myeloma
Hb Barts - gamma4 in defect in all 4 alpha genes

33. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Inc UCB
VWD
Sickle cell
Leukemoid reaction

34. What is the Ddx for for a macrocytic anemia
Nodular sclerosing
G6PD
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Inc UCB

35. Which substrates begin the heme synthesis pathway
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Glycine and succinyl - coa
Inc vasodiltion - inc perm - inc pain

36. What is the effected enzyme in acute intermittment porphyria?
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Africa = Jaw lesion - US = pelvis or abdomen
Bernard soulier

37. Wilm's tumor - RCC - HCC - hydronephrosis
Glanzmann's throbmasthenia
Dec synthesis of factors 1972 - protein C/S
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Contiguous

38. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Dec plasma volume
Heinz bodies - seen in alpha thal and G6PD
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

39. What do the labs show for TTP?
Ankryin - band 4.1 or spectrin
HbSS
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Schistocytes and inc LDH

40. hemolytic in a newborn - dec ATP and rigid RBCs
Hb Barts - gamma4 in defect in all 4 alpha genes
Pyruvate kinase def - extravascular
Hereditary elloptocytosis
G6PD

41. Why does B12 def cause neuro sx?
Aplastic anemia - pancytopenia
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
MGUS - monoclonal gammopathy of undetermined significance
B12 in fatty acid pathways leads to subacute combined degeneration

42. Is G6PD intravascular or extravascular
Varying shapes
Intravascular
IFN gama
Hereditary spherocytosis - G6PD - sickle cell

43. Who are the professional APCs?
Decrease EPO
Dendritic cells?
Sideroblastic anemia
VIII

44. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Beta thal
Dendritic cells?
40 to 75
Inc lymphoctes - and less RS cells

45. mother's antibodies attack fetal RBCs
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Incactivates II - VII - IX - X - XI - XII
Up - down - nl

46. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Blasts > 5%
Blood
Leukemia

47. What is the tx for sickle cell
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Hydroxyurea - bone marrow transplant
Nl PT - elevated PTT - intrinsic pathway defect
Uroporphyrinogen decarboxylase

48. What do labs show in ITP?
Bone marrow infiltration - myelofibrosis
VIII
Helps platelts adhere to endothelium
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

49. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Up - down - nl
Inc RBC - dec O2 sat - inc EPO
5- FU - AZT - hydroxyurea
Inducers of primary antibody response

50. What is the treatment for sideroblastic anemia
Both alpha and beta thal
Yes - via MHC II
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
B6 therapy (pyrodixine)