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Hemeonc

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Answer 50 questions in 15 minutes.
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1. Elderly - mature b cell tumor with filamentous - hairlike projections
Hairy cell leukemia
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
CLL (SLL without the peripheral lymphocytosis
Sickle cell

2. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
Antigen - antibody
Lung disease - congenital heart diseaes - and high altitude
40 to 75

3. hemolytic in a newborn - dec ATP and rigid RBCs
20 to 40
Bernard soulier
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Pyruvate kinase def - extravascular

4. What is the life span of a normal RBC
120 days
Imatinib - anti bcr abl
Petechiae
Fe def - thal - pb poison - sideroblastic anemia

5. What is the tx for aplastic anemia
Thrombotic thrombocytopenic purpura
Beta 4
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Mycosis fundgoides/Sezary syndrome

6. S-100 and CD1a with birbeck granules
ATIII def
Langerhans cell histiocytosis
Produce antibody - lots of RER and golgi
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

7. What does bradykinin do?
Inappropriate absolute with inc RBCs and EPO
1 to 6
Inc vasodiltion - inc perm - inc pain
Degrades fibrin mesh and converts C3 to C3a

8. What reveresible things can a sideroblastic anemia
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Liver disease - abetalipoproteinemia - acntho = spiny
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Lead - and EtOH

9. drug that inhibits ADP induced expression of GpIIb/IIIa
Ticlopidine/clopidogrel
Histaminase and arylsulfatase
Intravascular hemolysis
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 -

10. What does ADP do?
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Aplastic anemia - pancytopenia
Porphobilinogen - delta ALA - uroporphyrin
Helps platelts adhere to endothelium

11. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Pos
Intravascular hemolysis
Gp1b
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

12. How do platelet disorders present?
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Pyruvate kinase def - extravascular
T(12;21)
All

13. What is the therapy for the M3 variant?
Anemia of chronic disease - Aplastic anemia - kidney disease
Parvovirus
All trans retinoic acid
Factor V resistant to activated protein C's inhibition

14. How are plasma cells characterized?
All
Off center nuclues - clock face chromatin
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Inappropriate absolute with inc RBCs and EPO

15. What is the characteristic histologic finding in Hodgkin Lymphoma
20 to 40
CD15 and CD30 pos
Reed - sternberg cells
Porphobilinogen deaminase aka uroporphyrinogen I synthase

16. What virus is associated with 50% of Hodgkin lymphoma
EBV
B cells
Produce antibody - lots of RER and golgi
B6 therapy (pyrodixine)

17. What do labs show in ITP?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
TRAP (tartrate resistant acid phosphatase
Beta chain underproduced - asymptomatic - heterozygote
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

18. What CD molecules are on RS cells
VWD
CD15 and CD30 pos
Signs and sx of aplastic anemia
Fe def - thal - pb poison - sideroblastic anemia

19. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Off center nuclues - clock face chromatin
B12 def
VWF carries/protects factor VIII
Leukemoid reaction

20. Which drugs can cause macrocytic anemia?
5- FU - AZT - hydroxyurea
Yes - via MHC II
DIC - TTP/HUS - traumatic hemolysis
B6 therapy (pyrodixine)

21. Ddx for microcytic anemia
EBV
Ferrocheletase and ALA dehydrogenase
Positive osmootic fragility test and splenectomy
Fe def - thal - pb poison - sideroblastic anemia

22. What are the likely exposures of kids and adults for lead poisoning
Kids= exposure to lead paint - adults = battery - ammunition factory
G6PD
Sideroblastic anemia
Blood

23. Which substrates begin the heme synthesis pathway
Glycine and succinyl - coa
Malaria - Babesia
Ankryin - band 4.1 or spectrin
Beta thal

24. What is the pathogenesis of aplastic anemia with kidney disease
T(9;22) bcr abl
Decrease EPO
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Von Willebrand's disease

25. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Complications of sickle cell anemia
Inc large vWF multimers - inc platelet aggregation and thrombosis
Blasts > 5%
Extravascular

26. What are the extrinsic hemolytic normocytic anemias?
Up - down - nl
Protein S - cleaves and invactivates Va and VIIIa
...
Hb Barts - gamma4 in defect in all 4 alpha genes

27. What does decreasing heme do to ALA synthase activity?
Tissue
Myelofibrosis (marow is crying being its fibrosed'
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Inc

28. adults - auer rods - inc circulating myeblasts on peripheral smear
Dec
Def in factor IX
Porphobilinogen deaminase aka uroporphyrinogen I synthase
AML

29. What happens in beta thal major?
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
AB - no antibodies
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
VWF and fibrinogen

30. iron deficiency anemia - esophageal web - atrophic glossitis
Plummer - vinson syndrome
Hb Barts - gamma4 in defect in all 4 alpha genes
AB - no antibodies
All

31. What converts plasminogen to plasm and What does plasmin do?
No lytic bone lesions in WM
TPA - cleavage of fibrin mesh
Both alpha and beta thal
Her next Rh+ fetus

32. What is the Ddx for for a macrocytic anemia
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
EBV
Dec
B cells

33. What does the blood smear show in glanzmann's?
No platelet clumping
Megakaryocytes
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Mantle cell - older males

34. What does 'Neutrophils Like Making Everything Better' stand for?
Tissue factor converst the VII and VIIa
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Bleeding - malnutrition/absorption - inc demand pregs
Lysosomes

35. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
ATIII def
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
SLE - CLL - alpha methyldopa

36. What is monoclonal expansion without symptoms associated with multiple myeloma?
Mycosis fundgoides/Sezary syndrome
MGUS - monoclonal gammopathy of undetermined significance
All
ADP and Ca

37. What is hemophilia B?
Varying sizes
Complications of sickle cell anemia
M3 AML (acute promyelocytic leukemia)
Def in factor IX

38. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
B12/folate def
Macrohemorrhage - hemarthroses - easy bruising
DIC
Cromolyn sodium

39. What are the labs and tx for HS?
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
GpIIb/IIIa
Positive osmootic fragility test and splenectomy
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

40. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Down - up - down
Histaminase and arylsulfatase
120 days
Essential thrombocytosis

41. inc HbF on electrophoresis
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Anemia of chronic disease - Aplastic anemia - kidney disease
Both alpha and beta thal

42. What is the presenting scenario for TTP?
Adult T cell lymphoma - presents with cutaneous lesions
B12 def
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Blasts > 5%

43. What is HbH
Beta 4
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Blasts > 5%
Prothrombin gene mutation

44. Deficiency in vWF

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45. Who are the professional APCs?
Reed - sternberg cells
Glucose and heme - which inhibit ALA synthase
Dendritic cells?
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

46. What is appropriate absolyte polycythemia associated with
Lung disease - congenital heart diseaes - and high altitude
Off center nuclues - clock face chromatin
B symptoms - fever night sweats - weight loss
Hereditary spherocytosis

47. What causes renal papillary necrosis in sickle cell
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Complications of sickle cell anemia
Anemia of chronic disease - Aplastic anemia - kidney disease
Low O2 in papilla; can also get microhematuria from medullary infarcts

48. What are the four levels of alpha thal?
Porphyrias
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Imatinib - anti bcr abl
40 to 50

49. What is the defect in beta thal?
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Splicing sites and promotor sequences
Hairy cell leukemia
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

50. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Glucose and heme - which inhibit ALA synthase
Kids= exposure to lead paint - adults = battery - ammunition factory
Polycythemia vera
Folate def

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