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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. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Epoxide reductase - warfarin inhibits
40 to 75
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Sickle cell anemia

2. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Macrohemorrhage - hemarthroses - easy bruising
Complications of sickle cell anemia

3. What state is commonly associated with nonHod lymphoma
Extravascular hemolysis
Inc
HbSS
HIV or immunosupression

4. Wilm's tumor - RCC - HCC - hydronephrosis
Antigen - antibody
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Howell Jolly bodies

5. What activates the intrinsic pathway?
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Africa = Jaw lesion - US = pelvis or abdomen
Direct coombs - agglutinate if RBCs are coated with Ig
Collagen - BM - activated platelets

6. What do labs show in ITP?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Lysine for glutamate at position 6
SLE - CLL - alpha methyldopa
Blistering cutaneous photosens - most common porphyria

7. Ddx for microcytic anemia
Fe def - thal - pb poison - sideroblastic anemia
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
HIV or immunosupression
Inactivates it

8. Teardrop cell
Bone marrow infiltration - myelofibrosis
All
Cromolyn sodium
HbSS

9. What do you see on peripheral smear with sideroblastic anemia
Ringed sideronblasts with iron laden mitochondria
Multiple myeloma
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Down - down - up

10. What is the Ddx for a normocytic - normochromic anemia?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Anti Rh
Idiopathic thrombocytopenia (ITP)
Lung disease - congenital heart diseaes - and high altitude

11. What is factor V leidin?

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12. What substance accumulates in porphyria cutanea
2 to 10
Uroporphyrin (tea colored urine)
B12/folate def
Low in CML

13. What does decreasing heme do to ALA synthase activity?
Inc
Intrinsic - all factors except - VII - XIII
Beta chain underproduced - asymptomatic - heterozygote
Down - up - down

14. What does NAACP stand for - in regards to the causes of eosinophiia?
Glucose and heme - which inhibit ALA synthase
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Bernard soulier

15. What virus is associated with Burkitt lymphoma
EBV
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
No platelet clumping
Antigen - antibody

16. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
Lysine for glutamate at position 6
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
PGI2 - NO inc blood flow - dec platelet aggregation
DIC

17. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
CLL (SLL without the peripheral lymphocytosis
Gp1b
PGI2 - NO inc blood flow - dec platelet aggregation
Prothrombin gene mutation

18. anisocytosis
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Heinz bodies - seen in alpha thal and G6PD
8-10 days
Varying sizes

19. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
T(12;21)
Beta 4
ADP and Ca

20. philadelphia chromosome - blood looks like marrow
Protoporphyrin (blood)
CML
CLL (SLL without the peripheral lymphocytosis
Common a few days after oxidative stress in a pt with G6PD

21. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Adult T cell lymphoma - presents with cutaneous lesions
Up - down - up
Histaminase and arylsulfatase
Up - down - nl

22. Who are the professional APCs?
Multiple myeloma
Myelofibrosis (marow is crying being its fibrosed'
Dendritic cells?
No platelet clumping

23. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
Diffuse Large b cell lymphoma
Extrinsic - I - II - V - VII and X
Glanzmann's throbmasthenia
ATIII def

24. What do platelets release necessary for coagulation cascaed?
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Blood
CNS and testis
ADP and Ca

25. What symptoms are associated with Hodgkin lymphoma
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Ewing sarcoma
Inc HbF and dec HbS
B symptoms - fever night sweats - weight loss

26. What causes the jaundice in extravascular hemolysis
Inc UCB
DIC
Blod - tissue - MACS
Lysosomes

27. mother's antibodies attack fetal RBCs
Schistocytes and inc LDH
VIII
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
B12 def

28. What are some classic examples of extravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Multiple myeloma
Beta 4

29. Spherocyte
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Hereditary spherocytosis - autoimmune hemolysis
Inc lymphoctes - and less RS cells
Mantle cell lymphoma

30. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Valine for glutamate
Dec synthesis of factors 1972 - protein C/S
Imatinib - anti bcr abl
Idiopathic thrombocytopenia (ITP)

31. What is the therapy for CML (philly chrom)
VWF carries/protects factor VIII
Imatinib - anti bcr abl
Inc
Porphobilinogen deaminase aka uroporphyrinogen I synthase

32. How does the therapy for M3 vairant work?
Histaminase and arylsulfatase
No antigen - both antibodies
Induces differentiation of myeloblasts
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

33. What causes hydrops fetalis
Hb Barts - gamma4 in defect in all 4 alpha genes
Antigen - antibody
Leukemoid reaction
Low in CML

34. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
VWD
Parvovirus
Hodgkin
GpIIb/IIIa

35. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
SLE - CLL - alpha methyldopa
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Sickle cell

36. What percentage if WBCs are polys?
Glanzmann's throbmasthenia
Fibrinogen
Varying sizes
40 to 75

37. Which pathway and factors are tested in the PTT test
Ferrocheletase and ALA dehydrogenase
Varying sizes
Intrinsic - all factors except - VII - XIII
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

38. discrete tumor masses arising from lymph nodes
Protoporphyrin
Glycine and succinyl - coa
Lymphoma
Degrades fibrin mesh and converts C3 to C3a

39. What is the activation stage of platelet plug formation?
Nodular sclerosing
No lytic bone lesions in WM
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Hereditary spherocytosis

40. What is the tx for aplastic anemia
IgG - Warm is GREAT
Ringed sideronblasts with iron laden mitochondria
Langerhans cell histiocytosis
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

41. Where do B cells arise from - mature - and migrate to...
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Nl - up - nl
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

42. What causes renal papillary necrosis in sickle cell
Low O2 in papilla; can also get microhematuria from medullary infarcts
Common a few days after oxidative stress in a pt with G6PD
Antigen - antibody
Inc HbA2 on electrophoresis

43. What is the effect of ACE on bradykinin
Down - down - up
VWF and fibrinogen
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Inactivates it

44. Serum iron - transferrin - ferritin lab values for iron def anemia
Sickle cell
Pos
AB - no antibodies
Down - up - down

45. Elderly - mature b cell tumor with filamentous - hairlike projections
Deficiency in factor VIII
Inc vasodiltion - inc perm - inc pain
B6
Hairy cell leukemia

46. What are the azuraphilic granules in PMNs
Lysosomes
VWF and fibrinogen
Intrinsic - all factors except - VII - XIII
Inc UCB

47. What do the dense granules contain in platelets
Extravascular
Low O2 in papilla; can also get microhematuria from medullary infarcts
Paraprotein spike - monoclonal protein
ADP and Ca

48. What does plasmin do?
Degrades fibrin mesh and converts C3 to C3a
Hereditary elloptocytosis
Direct coombs - agglutinate if RBCs are coated with Ig
Collagen - BM - activated platelets

49. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Round densly staining nucleus with a small amount of pale cytoplasm
Nodular sclerosing
Varying sizes
G6PD

50. What are the four levels of alpha thal?
Sickle cell
Blasts > 5%
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Examples of oxidative stress