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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. hemolytic in a newborn - dec ATP and rigid RBCs
Pyruvate kinase def - extravascular
1 to 6
Dec plasma volume
Off center nuclues - clock face chromatin

2. What does ADP do?
Helps platelts adhere to endothelium
DIC
Histaminase and arylsulfatase
Protoporphyrin (blood)

3. What activates the intrinsic pathway?
Collagen - BM - activated platelets
Paraprotein spike - monoclonal protein
Dec
Sickle cell

4. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Leukemoid reaction
G6PD
Signs and sx of aplastic anemia

5. Blood type A
Inc RBC - dec O2 sat - inc EPO
T(9;22) bcr abl
A antigen and B antibodies
ATIII def

6. What causes hydrops fetalis
Beta 4
Schistocytes and inc LDH
Ferrocheletase and ALA dehydrogenase
Hb Barts - gamma4 in defect in all 4 alpha genes

7. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
ATIII def
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Up - down - up
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

8. What is the mutation in HbC
Megakaryocytes
90% anearobically from glucose to lactate - 10% from HMP shunt
Def in factor IX
Lysine for glutamate at position 6

9. iron deficiency anemia - esophageal web - atrophic glossitis
T(9;22) bcr abl
Plummer - vinson syndrome
VIII
Off center nuclues - clock face chromatin

10. inc LDH - jaundice
Von Willebrand's disease
Extravascular hemolysis
T(12;21)
VIII

11. What indicated a good prognosis in Hodgkin lymphoma?
Inc lymphoctes - and less RS cells
B symptoms - fever night sweats - weight loss
Macrohemorrhage - hemarthroses - easy bruising
Direct coombs - agglutinate if RBCs are coated with Ig

12. What are the variants of the RS cells
T(9;22) bcr abl
Beta 4
Lacunar in nodular sclerosing variant
No antigen - both antibodies

13. Where do B cells arise from - mature - and migrate to...
Dec plasma volume
No lytic bone lesions in WM
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Malaria - Babesia

14. What do labs show in DIC?
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Pyruvate kinase def - extravascular
G6PD
Glanzmann's throbmasthenia

15. Where does All spread
Deficiency in factor VIII
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
G6PD
CNS and testis

16. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Factor V Leiden
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Schistocytes and inc LDH
Direct coombs - agglutinate if RBCs are coated with Ig

17. What chromosomal translocation is associated with a better prognosis in All
MGUS - monoclonal gammopathy of undetermined significance
Dec synthesis of factors 1972 - protein C/S
Splicing sites and promotor sequences
T(12;21)

18. What is factor V leidin?

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19. What does STOP Making New Thrombi stand for
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Hodgkin
CML to AML or All

20. How are plasma cells characterized?
Off center nuclues - clock face chromatin
Intrinsic - all factors except - VII - XIII
CD15 and CD30 pos
B symptoms - fever night sweats - weight loss

21. What substance prevents mast cells degranulation?
Exposed collagen upon endothelial damage
Cromolyn sodium
Mantle cell - older males
Mantle cell lymphoma

22. What percentage if WBCs are polys?
40 to 75
DDAVP (desmopressin) which releases stored vWF stored in endothelium
CML to AML or All
CD15 and CD30 pos

23. What cell is primarily involved in non Hod lymph
Fibrinogen
Inc HbA2 on electrophoresis
B cells
Helps platelts adhere to endothelium

24. What is the treatment to prevent a woman from forming anti Rh antibody?
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Blasts > 5%
Rhogam - Rh antigen immunoglobulin
Easy gas exchange

25. What are the anti aggregation factors?
Prothrombin gene mutation
PGI2 - NO inc blood flow - dec platelet aggregation
CML
AB - no antibodies

26. What causes the physiologic chloride shift and What does the chloride shift do?
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Hereditary elloptocytosis
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
T(12;21)

27. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Ankryin - band 4.1 or spectrin
Polycythemia vera
Bone marrow infiltration - myelofibrosis
Adult T cell lymphoma - presents with cutaneous lesions

28. What are the etiologies of folate def
Blood
TPA - cleavage of fibrin mesh
Follicular lymphoma - indolent course
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

29. What does increasing heme do to ALA synthase activity
Heinz bodies - seen in alpha thal and G6PD
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Off center nuclues - clock face chromatin
Dec

30. What do you see a starry sky appearance in Burkitt
Idiopathic thrombocytopenia (ITP)
Bleeding - malnutrition/absorption - inc demand pregs
Sheets of lymphocytes interspersed with macs
VIII

31. What is the activation stage of platelet plug formation?
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 -
Extravascular hemolysis
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Howell Jolly bodies

32. What are the azuraphilic granules in PMNs
A antigen and B antibodies
Anti Rh
Lysosomes
Prothrombin gene mutation

33. What percentage of WBCs are basophils - and What is found in their basophilic granules?
HbSS
ATIII def
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

34. S-100 and CD1a with birbeck granules
Dec synthesis of factors 1972 - protein C/S
Positive osmootic fragility test and splenectomy
Adult T cell lymphoma - presents with cutaneous lesions
Langerhans cell histiocytosis

35. What are the likely exposures of kids and adults for lead poisoning
20 to 40
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Kids= exposure to lead paint - adults = battery - ammunition factory
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

36. Megakaryocytosis
SLE - CLL - alpha methyldopa
Inc UCB
No antigen - both antibodies
Essential thrombocytosis

37. t(11:22)
M3 AML (acute promyelocytic leukemia)
CNS and testis
CML
Ewing sarcoma

38. What do the dense granules contain in platelets
Incactivates II - VII - IX - X - XI - XII
20 to 40
Inc HbF and dec HbS
ADP and Ca

39. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Low in CML
VIII
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Leukemoid reaction

40. What is the mutation in HbS
CLL (SLL without the peripheral lymphocytosis
Parvovirus
Valine for glutamate
Inc RBC - dec O2 sat - inc EPO

41. Drug that inhbits the GpIIb/IIIa directly
Inappropriate absolute with inc RBCs and EPO
Uroporphyrin (tea colored urine)
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Abciximab

42. Where do you see hypersegmented polys?
Uroporphyrin (tea colored urine)
Hb Barts - gamma4 in defect in all 4 alpha genes
MGUS - monoclonal gammopathy of undetermined significance
B12/folate def

43. Where to T cells arise and mature - and migrate to...
G6PD
Macrohemorrhage - hemarthroses - easy bruising
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Intrinsic - all factors except - VII - XIII

44. What is the characteristic histologic finding in Hodgkin Lymphoma
Diffuse Large b cell lymphoma
Reed - sternberg cells
Eleveated PT - PTT
Beta chain underproduced - asymptomatic - heterozygote

45. anisocytosis
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Ewing sarcoma
Porphyrias
Varying sizes

46. tennis rackets on EM
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Birbeck granules
EBV
TXA2 - dec blood flow - inc platelet aggregation

47. What is the affected enzyme in acute intermittment porphyria
Blistering cutaneous photosens - most common porphyria
B6
Sickle cell
Porphobilinogen - delta ALA - uroporphyrin

48. Mutation in 3' untranslated region associated with venous clots
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Prothrombin gene mutation
Down - up - down
GpIIb/IIIa

49. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
TXA2 - dec blood flow - inc platelet aggregation
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Protein S - cleaves and invactivates Va and VIIIa
CLL (SLL without the peripheral lymphocytosis

50. Is HS extravascular or intravascular?
B6 therapy (pyrodixine)
Extravascular
Easy gas exchange
AB - no antibodies