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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. What is appropriate absolute polycythemia
Acts to stabilize platelet plug
CNS and testis
Gp1b
Inc RBC - dec O2 sat - inc EPO

2. What are some causes of warm agglutinin autoimmune hemolytic anemia?
SLE - CLL - alpha methyldopa
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Low O2 in papilla; can also get microhematuria from medullary infarcts
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

3. What is the mutation in HbC
CNS and testis
Inc HbA2 on electrophoresis
Lysine for glutamate at position 6
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

4. What is the clinical picture of hemophilia A or B
Macrohemorrhage - hemarthroses - easy bruising
Tissue factor converst the VII and VIIa
TPA - cleavage of fibrin mesh
Polycythemia vera

5. t(11;14)
Ankryin - band 4.1 or spectrin
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Bone marrow infiltration - myelofibrosis
Mantle cell lymphoma

6. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Leukemia
Helps platelts adhere to endothelium
Liver disease - abetalipoproteinemia - acntho = spiny
Inc UCB

7. RBCs damagaed from passing through obstructed - narrowed vessel lumina
MAHA
Factor V resistant to activated protein C's inhibition
8-10 days
Contiguous

8. What substance prevents mast cells degranulation?
Lead - and EtOH
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Cromolyn sodium
Intravascular

9. What is the characteristic spread of Hodgkin Lymphoma
Hereditary spherocytosis
Fc
Contiguous
Uroporphyrinogen decarboxylase

10. Elliptocyte
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Hereditary elloptocytosis
Reed - sternberg cells
B cells

11. Macro - ovalocyte
MAHA
Lymphoma
Intrinsic - all factors except - VII - XIII
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

12. Where do B cells arise from - mature - and migrate to...
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Follicular lymphoma - indolent course
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Both alpha and beta thal

13. Which substrates begin the heme synthesis pathway
Beta thal
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Signs and sx of aplastic anemia
Glycine and succinyl - coa

14. What is the pathogenesis of TTP?
20 to 40
AML
Schistocytes and inc LDH
Inc large vWF multimers - inc platelet aggregation and thrombosis

15. What symptoms are associated with Hodgkin lymphoma
Peroxidase
Idiopathic thrombocytopenia (ITP)
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
B symptoms - fever night sweats - weight loss

16. tennis rackets on EM
Schistocytes and inc LDH
Birbeck granules
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Dec

17. Basophilic nuclear remnants fonud in RBCs
Howell - Jolly bodies - hypo/asplenia
Uroporphyrinogen decarboxylase
Lysine for glutamate at position 6
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

18. normal RBCs added to patient's serum
Indirect coombs - agglutinate if serum anti RBC surface Ig
Ankryin - band 4.1 or spectrin
B12 in fatty acid pathways leads to subacute combined degeneration
VWD

19. Deficiency in vWF

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20. What is the life spance of a platelet?
8-10 days
Ringed sideronblasts with iron laden mitochondria
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
T(9;22) bcr abl

21. What role does antithrombin play?
EBV
Lung disease - congenital heart diseaes - and high altitude
Lysosomes
Incactivates II - VII - IX - X - XI - XII

22. Drug that inhbits the GpIIb/IIIa directly
B6 therapy (pyrodixine)
Nodular sclerosing
Abciximab
Dec synthesis of factors 1972 - protein C/S

23. What are some classic examples of extravascular hemolysis
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell
Blistering cutaneous photosens - most common porphyria
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

24. What finding you do you see in patients after splenectomy
Howell Jolly bodies
Inc HbA2 on electrophoresis
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Blistering cutaneous photosens - most common porphyria

25. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Incactivates II - VII - IX - X - XI - XII
Indirect coombs - agglutinate if serum anti RBC surface Ig
Howell Jolly bodies
Polycythemia vera

26. What virus can cause an aplastic crisis in pts with HS?
Parvovirus
Inactivates it
Heinz bodies - seen in alpha thal and G6PD
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

27. Which factor does vWF carry/protect
VIII
Aplastic anemia - pancytopenia
Bleeding - malnutrition/absorption - inc demand pregs
Inc HbF and dec HbS

28. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Anemia of chronic disease - Aplastic anemia - kidney disease
Sickle cell
Malaria - Babesia

29. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Protein C or S deficiency
Exposed collagen upon endothelial damage
Epoxide reductase - warfarin inhibits
MGUS - monoclonal gammopathy of undetermined significance

30. Where are monocytes typically found - where do they go - and What do the differentiate into?
Blod - tissue - MACS
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Inc lymphoctes - and less RS cells
Inc serum iron - normal TIBC - inc ferratin

31. What substance accumulates in porphyria cutanea
G6PD
Uroporphyrin (tea colored urine)
SLE - CLL - alpha methyldopa
Easy gas exchange

32. anti - Ig antibody added to patients RBCs;
TPA - cleavage of fibrin mesh
Ferrocheletase and ALA dehydrogenase
Direct coombs - agglutinate if RBCs are coated with Ig
Low O2 in papilla; can also get microhematuria from medullary infarcts

33. Which immunoglobulin is involved in warm agglutination?
T(12;21)
Dec
IgG - Warm is GREAT
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

34. What activates protein C and What does activated protein C do?
Fc
5- FU - AZT - hydroxyurea
Valine for glutamate
Protein S - cleaves and invactivates Va and VIIIa

35. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Epoxide reductase - warfarin inhibits
Signs and sx of aplastic anemia
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 -
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

36. Ringed sideroblasts
Sideroblastic anemia
EBV
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

37. What are the four levels of alpha thal?
Aplastic anemia - pancytopenia
Blood
Protein C or S deficiency
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

38. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
No lytic bone lesions in WM
VWD
TPA - cleavage of fibrin mesh
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

39. Bone pain plus anemia
Multiple myeloma
CML to AML or All
TXA2 - dec blood flow - inc platelet aggregation
Tissue factor converst the VII and VIIa

40. Ddx for microcytic anemia
Paroxysmal nocturnal hemoglobinuria
Burkitt lymphoma
Positive osmootic fragility test and splenectomy
Fe def - thal - pb poison - sideroblastic anemia

41. bcl -2 t(14;18) adults
Follicular lymphoma - indolent course
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Burkitt lymphoma
Bone marrow infiltration - myelofibrosis

42. What activates the fibrinolytic pathway?
Down - up - down
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
B6 therapy (pyrodixine)

43. What is the age breakdown for hodgkins
Bimodal - men>women - except for nodular sclerosing type
ATIII def
Protoporphyrin (blood)
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

44. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
B12 in fatty acid pathways leads to subacute combined degeneration
Hereditary elloptocytosis
Epoxide reductase - warfarin inhibits
Extravascular hemolysis

45. How are the nucleus and the cytoplasm characterized for lymphocytes
Ferrocheletase and ALA dehydrogenase
120 days
Sideroblastic anemia
Round densly staining nucleus with a small amount of pale cytoplasm

46. From what cells are platelets derived from
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Megakaryocytes
Sheets of lymphocytes interspersed with macs
Protoporphyrin

47. What are the labs and tx for HS?
B symptoms - fever night sweats - weight loss
Helps platelts adhere to endothelium
Degrades fibrin mesh and converts C3 to C3a
Positive osmootic fragility test and splenectomy

48. What is the coombs test results in AIHA?
Pos
CLL (SLL without the peripheral lymphocytosis
Ferrocheletase and ALA dehydrogenase
DIC - TTP/HUS - traumatic hemolysis

49. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Yes - via MHC II
Off center nuclues - clock face chromatin
ATIII def

50. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
SLE - CLL - alpha methyldopa
Off center nuclues - clock face chromatin
B12 def
Lysosomes