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Hemeonc

Instructions:

Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
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This is a study tool. The 3 wrong answers for each question are randomly chosen from answers to other questions. So, you might find at times the answers obvious, but you will see it re-enforces your understanding as you take the test each time.

1. Is HS extravascular or intravascular?
Low O2 in papilla; can also get microhematuria from medullary infarcts
Extravascular
EBV
All

2. Is G6PD intravascular or extravascular
Intravascular
40 to 50
Low in CML
TPA - cleavage of fibrin mesh

3. What percentage of WBCs are eosinophils?
Def in factor IX
Pyruvate kinase def - extravascular
1 to 6
Mantle cell lymphoma

4. drug that inhibits ADP induced expression of GpIIb/IIIa
Ticlopidine/clopidogrel
Bone marrow infiltration - myelofibrosis
Ringed sideronblasts with iron laden mitochondria
Prothrombin gene mutation

5. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Essential thrombocytosis
Inappropriate absolute with inc RBCs and EPO
Follicular lymphoma - indolent course
Complications of sickle cell anemia

6. Who are the professional APCs?
Def in factor IX
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
TPA - cleavage of fibrin mesh
Dendritic cells?

7. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Collagen - BM - activated platelets
Nl - up - nl
Kids= exposure to lead paint - adults = battery - ammunition factory
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

8. t(11;14)
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Up - down - up
Mantle cell lymphoma
Abciximab

9. What are the age ranges for the various leukemias
Spleen
Her next Rh+ fetus
Extrinsic - I - II - V - VII and X
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

10. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Helminth infections major basic protein
Howell - Jolly bodies - hypo/asplenia
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
VWD

11. iron deficiency anemia - esophageal web - atrophic glossitis
Direct coombs - agglutinate if RBCs are coated with Ig
Plummer - vinson syndrome
2 to 10
Epoxide reductase - warfarin inhibits

12. What is the treatment to prevent a woman from forming anti Rh antibody?
Hairy cell leukemia
Contiguous
Rhogam - Rh antigen immunoglobulin
Uroporphyrin (tea colored urine)

13. Spherocyte
Histamine - heparin - and eosinohil chemotactic factors
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 -
CNS and testis
Hereditary spherocytosis - autoimmune hemolysis

14. What do labs show in ITP?
B cells
Inducers of primary antibody response
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Hb Barts - gamma4 in defect in all 4 alpha genes

15. When do you see MAHA?
Yes - via MHC II
IFN gama
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
IgG - Warm is GREAT

16. t(15;17)
Protein S - cleaves and invactivates Va and VIIIa
M3 AML (acute promyelocytic leukemia)
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

17. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Inc RBC - dec O2 sat - inc EPO
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

18. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Multiple myeloma
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Polycythemia vera
5- FU - AZT - hydroxyurea

19. What is the characteristic histologic finding in Hodgkin Lymphoma
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Porphobilinogen - delta ALA - uroporphyrin
Reed - sternberg cells

20. What do platelets interact with to form a hemostatic plug
Off center nuclues - clock face chromatin
Heinz bodies - seen in alpha thal and G6PD
Fibrinogen
CLL (SLL without the peripheral lymphocytosis

21. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Hereditary spherocytosis - autoimmune hemolysis
Heinz bodies - seen in alpha thal and G6PD
120 days
ADP and Ca

22. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Down - down - up
EBV
Porphobilinogen - delta ALA - uroporphyrin
Sickle cell anemia

23. What is the treatment for sideroblastic anemia
B6 therapy (pyrodixine)
HIV or immunosupression
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Langerhans cells

24. What are the main associations with multiple myeloma?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Inc RBC - dec O2 sat - inc EPO
Low in CML
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

25. Ddx for microcytic anemia
Fe def - thal - pb poison - sideroblastic anemia
Reed - sternberg cells
Petechiae
VWF carries/protects factor VIII

26. What is the philadelphia chromosome
T(9;22) bcr abl
Peroxidase
Lysosomes
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

27. Which pathway and factors are tested in the PTT test
Intrinsic - all factors except - VII - XIII
Lysine for glutamate at position 6
Positive osmootic fragility test and splenectomy
Histamine - heparin - and eosinohil chemotactic factors

28. Where does All spread
CNS and testis
Collagen - BM - activated platelets
Porphyrias
GpIIb/IIIa

29. What substances are released in mast cell degranulation
Histamine - heparin - and eosinohil chemotactic factors
Ticlopidine/clopidogrel
Intravascular hemolysis
Beta chain underproduced - asymptomatic - heterozygote

30. What does antithrombin do and What activates it?
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Schistocytes - helmet cells
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

31. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Folate def
Hb Barts - gamma4 in defect in all 4 alpha genes
Porphobilinogen - delta ALA - uroporphyrin
Leukemia

32. S-100 and CD1a with birbeck granules
Parvovirus
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Reed - sternberg cells
Langerhans cell histiocytosis

33. What are dendritic cells called in the skin?
Langerhans cells
Complications of sickle cell anemia
Contiguous
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

34. What is the life spance of a platelet?
8-10 days
Dec
ATIII def
Yes - via MHC II

35. What does increasing heme do to ALA synthase activity
Dec
Incactivates II - VII - IX - X - XI - XII
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Her next Rh+ fetus

36. What do auer rods stain with
Peroxidase
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Gp1b

37. What are the presenting symptoms of acute intermittent porphyria
Sideroblastic anemia
CML to AML or All
Howell - Jolly bodies - hypo/asplenia
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

38. Blood type A
ATIII def
Bone marrow infiltration - myelofibrosis
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
A antigen and B antibodies

39. What reveresible things can a sideroblastic anemia
Lead - and EtOH
Polycythemia vera
Epoxide reductase - warfarin inhibits
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

40. Eosinophils are highly phagocytic For what kind of complex?
Her next Rh+ fetus
DIC
Antigen - antibody
Intravascular

41. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Adult T cell lymphoma - presents with cutaneous lesions
Porphyrias
CLL (SLL without the peripheral lymphocytosis
Paroxysmal nocturnal hemoglobinuria

42. What is the effect of ACE on bradykinin
Inactivates it
Idiopathic thrombocytopenia (ITP)
Hereditary spherocytosis
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

43. Can B cells function as APCs?
Induces differentiation of myeloblasts
Yes - via MHC II
Idiopathic thrombocytopenia (ITP)
Ankryin - band 4.1 or spectrin

44. What is the activation stage of platelet plug formation?
Megakaryocytes
Helminth infections major basic protein
Schistocytes and inc LDH
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

45. hemolytic in a newborn - dec ATP and rigid RBCs
T(12;21)
Decrease EPO
Pyruvate kinase def - extravascular
Bone marrow infiltration - myelofibrosis

46. Plasma cell neoplasm
G6PD
90% anearobically from glucose to lactate - 10% from HMP shunt
Multiple myeloma
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

47. Which substrates begin the heme synthesis pathway
SLE - CLL - alpha methyldopa
Glycine and succinyl - coa
Spleen
Factor V resistant to activated protein C's inhibition

48. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Pos
Sickle cell
Hereditary spherocytosis
Aplastic anemia - pancytopenia

49. Which pathway and factorrs are tested by the PT coag test
B12/folate def
Extrinsic - I - II - V - VII and X
Epoxide reductase - warfarin inhibits
Heinz bodies - seen in alpha thal and G6PD

50. What converts plasminogen to plasm and What does plasmin do?
Dec synthesis of factors 1972 - protein C/S
TPA - cleavage of fibrin mesh
No antigen - both antibodies
Lysine for glutamate at position 6