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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. Sickle cell
Contiguous
Sickle cell anemia
Lymphoma
Epoxide reductase - warfarin inhibits

2. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Sideroblastic anemia
Africa = Jaw lesion - US = pelvis or abdomen
Hereditary spherocytosis
1 to 6

3. What does ectopic EPO produce
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Nl PT - elevated PTT - intrinsic pathway defect
Inappropriate absolute with inc RBCs and EPO
Blod - tissue - MACS

4. What role does antithrombin play?
Polycythemia vera
Hereditary spherocytosis
Incactivates II - VII - IX - X - XI - XII
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

5. What is the pathogenesis of sickle cell
Fc
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Polycythemia vera

6. Where are mast cells found
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Porphyrias
Inc serum iron - normal TIBC - inc ferratin
Tissue

7. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Mantle cell - older males
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Examples of oxidative stress
Up - down - nl

8. What does bradykinin do?
Inc vasodiltion - inc perm - inc pain
120 days
Plummer - vinson syndrome
VWF and fibrinogen

9. What percentage of WBCs are basophils - and What is found in their basophilic granules?
Inc large vWF multimers - inc platelet aggregation and thrombosis
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
CNS and testis

10. What CD molecules are on RS cells
CD15 and CD30 pos
Helminth infections major basic protein
Glycine and succinyl - coa
Direct coombs - agglutinate if RBCs are coated with Ig

11. What happens in betal thal minor?
Megakaryocytes
Lead - and EtOH
Diffuse Large b cell lymphoma
Beta chain underproduced - asymptomatic - heterozygote

12. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Mantle cell lymphoma
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
EBV
Up - down - nl

13. What is the coombs test results in AIHA?
Pos
Protein C or S deficiency
Varying shapes
Deficiency in factor VIII

14. What does the large SA:volume ratio in RBCs help facilitate?
Abciximab
Easy gas exchange
Hereditary spherocytosis - G6PD - sickle cell
No platelet clumping

15. Serum iron - transferrin - ferritin lab values for hemochromatosis
Ringed sideronblasts with iron laden mitochondria
Leukemia
Hydroxyurea - bone marrow transplant
Up - down - up

16. What do plasma cells do
Hodgkin
Produce antibody - lots of RER and golgi
HIV or immunosupression
Birbeck granules

17. what proteins can be defective in HS?
Ankryin - band 4.1 or spectrin
ADP and Ca
Extravascular
Pyruvate kinase def - extravascular

18. What is a blast crisis
Porphobilinogen - delta ALA - uroporphyrin
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Inducers of primary antibody response
CML to AML or All

19. Is G6PD intravascular or extravascular
B cells
Sideroblastic anemia
Intravascular
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

20. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Histaminase and arylsulfatase
Fe def - thal - pb poison - sideroblastic anemia
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Collagen - BM - activated platelets

21. What is the affected enzyme in lead poisoning
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
VIII
Abciximab
Ferrocheletase and ALA dehydrogenase

22. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Aplastic anemia - pancytopenia
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Protoporphyrin
Signs and sx of aplastic anemia

23. What is monoclonal expansion without symptoms associated with multiple myeloma?
B6 therapy (pyrodixine)
MGUS - monoclonal gammopathy of undetermined significance
Tissue
HIV or immunosupression

24. What causes renal papillary necrosis in sickle cell
Low O2 in papilla; can also get microhematuria from medullary infarcts
2 to 10
Hodgkin
Macrohemorrhage - hemarthroses - easy bruising

25. What do you see a starry sky appearance in Burkitt
5- FU - AZT - hydroxyurea
No platelet clumping
Lacunar in nodular sclerosing variant
Sheets of lymphocytes interspersed with macs

26. What is the pathogenesis of aplastic anemia with kidney disease
T(9;22) bcr abl
Decrease EPO
VWD
Porphobilinogen deaminase aka uroporphyrinogen I synthase

27. Fava beans - sufla drugs - infectinos
Low in CML
Examples of oxidative stress
Leukemoid reaction
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

28. CD5+ - poor prognosis - t(11;14)
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Tissue
Mantle cell - older males
Schistocytes - helmet cells

29. Who are the professional APCs?
1 to 6
Dendritic cells?
Histaminase and arylsulfatase
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

30. What portion of IgE can mast cells bind
Fc
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Produce antibody - lots of RER and golgi
B cells

31. discrete tumor masses arising from lymph nodes
Lymphoma
Anemia of chronic disease - Aplastic anemia - kidney disease
No lytic bone lesions in WM
Epoxide reductase - warfarin inhibits

32. What are some causes of cold agglutinin anemia
CML to AML or All
Follicular lymphoma - indolent course
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Sheets of lymphocytes interspersed with macs

33. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Abciximab
They bind vWF via GpIb
Sheets of lymphocytes interspersed with macs
Hereditary spherocytosis

34. What are the presenting symptoms of porphyria cutanea tarda
1 to 6
Blistering cutaneous photosens - most common porphyria
Diffuse Large b cell lymphoma
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

35. Ddx for microcytic anemia
Exposed collagen upon endothelial damage
Fe def - thal - pb poison - sideroblastic anemia
Inducers of primary antibody response
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

36. What does STOP Making New Thrombi stand for
Decrease EPO
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
1 to 6

37. What is the life span of a normal RBC
Inc vasodiltion - inc perm - inc pain
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
120 days

38. What is appropriate absolute polycythemia
Peroxidase
Inc RBC - dec O2 sat - inc EPO
B12/folate def
M3 AML (acute promyelocytic leukemia)

39. Acanthocyte (spur cell)
Liver disease - abetalipoproteinemia - acntho = spiny
...
EBV
Burkitt lymphoma

40. Deficiency in GpIIb/IIIa

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41. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
B cells
Histamine - heparin - and eosinohil chemotactic factors
Down - down - up
Splicing sites and promotor sequences

42. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Ferrocheletase and ALA dehydrogenase
Leukemoid reaction
Intravascular
Down - up - down

43. RBCs damagaed from passing through obstructed - narrowed vessel lumina
Polycythemia vera
Kids= exposure to lead paint - adults = battery - ammunition factory
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
MAHA

44. Upregulated growth of leukocytes in bone marro
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Blasts > 5%
Leukemia
Plummer - vinson syndrome

45. What is the tx for sickle cell
Hydroxyurea - bone marrow transplant
Reed - sternberg cells
Langerhans cell histiocytosis
IFN gama

46. Mutation in 3' untranslated region associated with venous clots
Prothrombin gene mutation
DIC
Hereditary elloptocytosis
AML

47. Plasma cell neoplasm
Epoxide reductase - warfarin inhibits
Beta 4
Idiopathic thrombocytopenia (ITP)
Multiple myeloma

48. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Hereditary spherocytosis - autoimmune hemolysis
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Sideroblastic anemia
Paroxysmal nocturnal hemoglobinuria

49. What is the treatment to prevent a woman from forming anti Rh antibody?
Liver disease - abetalipoproteinemia - acntho = spiny
Off center nuclues - clock face chromatin
Common a few days after oxidative stress in a pt with G6PD
Rhogam - Rh antigen immunoglobulin

50. Teardrop cell
Howell - Jolly bodies - hypo/asplenia
Bone marrow infiltration - myelofibrosis
Def in factor IX
Low in CML