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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. When do you see MAHA?
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Varying sizes
Sheets of lymphocytes interspersed with macs
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

2. What is relative polycythemia
AB - no antibodies
B12 def
Dec plasma volume
Inc UCB

3. What activates protein C and What does activated protein C do?
Protein S - cleaves and invactivates Va and VIIIa
Inducers of primary antibody response
Protoporphyrin
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

4. What indicated a good prognosis 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)
Cromolyn sodium
Inc lymphoctes - and less RS cells
Up - down - nl

5. Back pain - hemoglobinuria
Plasma cell
Common a few days after oxidative stress in a pt with G6PD
T(9;22) bcr abl
Beta 4

6. What does ADP do?
Helps platelts adhere to endothelium
Paroxysmal nocturnal hemoglobinuria
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Ewing sarcoma

7. Conditions of defective heme synthesis leading to accumulation of heme precurors
B12/folate def
Porphyrias
Lysosomes
Schistocytes and inc LDH

8. normal RBCs added to patient's serum
Mycosis fundgoides/Sezary syndrome
Indirect coombs - agglutinate if serum anti RBC surface Ig
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Parvovirus

9. What symptoms are associated with Hodgkin lymphoma
90% anearobically from glucose to lactate - 10% from HMP shunt
Alpha thal - asian and african american
B symptoms - fever night sweats - weight loss
Uroporphyrin (tea colored urine)

10. Mutation in 3' untranslated region associated with venous clots
Prothrombin gene mutation
1 to 6
Helps platelts adhere to endothelium
Induces differentiation of myeloblasts

11. Which drugs can cause macrocytic anemia?
Inappropriate absolute with inc RBCs and EPO
Alpha thal - asian and african american
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
5- FU - AZT - hydroxyurea

12. What are some causes of cold agglutinin anemia
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Leukemia

13. philadelphia chromosome - blood looks like marrow
ADP and Ca
IgG - Warm is GREAT
CML
Protoporphyrin

14. How does vWD cause elevated PTT?
Aplastic anemia - pancytopenia
VWF carries/protects factor VIII
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

15. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Low in CML
Contiguous
Hodgkin
AML

16. What the alpha granules contain in platelets?
Bernard soulier
VWF and fibrinogen
Langerhans cell histiocytosis
Plummer - vinson syndrome

17. Where are mast cells found
Tissue
Idiopathic thrombocytopenia (ITP)
No antigen - both antibodies
CML to AML or All

18. What converts plasminogen to plasm and What does plasmin do?
Incactivates II - VII - IX - X - XI - XII
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Pyruvate kinase def - extravascular
TPA - cleavage of fibrin mesh

19. What is contained within the azurophilic granules of PMNs
Inc large vWF multimers - inc platelet aggregation and thrombosis
Extravascular hemolysis
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
CLL (SLL without the peripheral lymphocytosis

20. Blood type A
A antigen and B antibodies
No antigen - both antibodies
AB - no antibodies
Eleveated PT - PTT

21. What do you see in vit K def
Exposed collagen upon endothelial damage
Schistocytes and inc LDH
Eleveated PT - PTT
Diffuse Large b cell lymphoma

22. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Sickle cell anemia
Adult T cell lymphoma - presents with cutaneous lesions
20 to 40
Signs and sx of aplastic anemia

23. Which cell is neoplastic in multiple myeloma
Blasts > 5%
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Plasma cell

24. poikilocytosis
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
ADP and Ca
Easy gas exchange
Varying shapes

25. hemolytic in a newborn - dec ATP and rigid RBCs
Examples of oxidative stress
Pyruvate kinase def - extravascular
Produce antibody - lots of RER and golgi
Peroxidase

26. What does hairy cell leukemia stain with
TRAP (tartrate resistant acid phosphatase
Antigen - antibody
Inducers of primary antibody response
Polycythemia vera

27. What are the extrinsic hemolytic normocytic anemias?
EBV
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
...
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

28. What role does antithrombin play?
A antigen and B antibodies
Hereditary spherocytosis
Incactivates II - VII - IX - X - XI - XII
Myelofibrosis (marow is crying being its fibrosed'

29. What is appropriate absolyte polycythemia associated with
Lung disease - congenital heart diseaes - and high altitude
Plummer - vinson syndrome
Hereditary spherocytosis - G6PD - sickle cell
Histamine - heparin - and eosinohil chemotactic factors

30. t(15;17)
G6PD
Rhogam - Rh antigen immunoglobulin
M3 AML (acute promyelocytic leukemia)
Lysosomes

31. What is the are the presenting symptoms of lead poisoning in kids and adults
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
SLE - CLL - alpha methyldopa
VWF and fibrinogen

32. What are the four levels of alpha thal?
No lytic bone lesions in WM
Histamine - heparin - and eosinohil chemotactic factors
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
M3 AML (acute promyelocytic leukemia)

33. What do you see in peripheral smear in a pt with G6PD?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Langerhans cell histiocytosis
Bite cells and Heinz bodies
Orotic aciduria

34. mother's antibodies attack fetal RBCs
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
T(9;22) bcr abl
Essential thrombocytosis
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

35. How does the therapy for M3 vairant work?
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Induces differentiation of myeloblasts
Dec
Plummer - vinson syndrome

36. What does STOP Making New Thrombi stand for
AML
Inducers of primary antibody response
Factor V resistant to activated protein C's inhibition
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

37. What is the characteristic lab finding on electrophoresis
Incactivates II - VII - IX - X - XI - XII
Paraprotein spike - monoclonal protein
Extravascular
90% anearobically from glucose to lactate - 10% from HMP shunt

38. What does antithrombin do and What activates it?
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Blood
TRAP (tartrate resistant acid phosphatase

39. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
PGI2 - NO inc blood flow - dec platelet aggregation
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
ATIII def
Malaria - Babesia

40. X linked - dec glutathione inc RBC susceptibility to oxidative stress
5- FU - AZT - hydroxyurea
Petechiae
B symptoms - fever night sweats - weight loss
G6PD

41. defect in alpha globin gene - dec alpha globin synth - disease and populations?
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Bite cells and Heinz bodies
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Alpha thal - asian and african american

42. What virus can cause an aplastic crisis in pts with HS?
Howell - Jolly bodies - hypo/asplenia
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Up - down - nl
Parvovirus

43. What is the treatment for acute intermittent porphyria
Inc HbA2 on electrophoresis
Glucose and heme - which inhibit ALA synthase
TPA - cleavage of fibrin mesh
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

44. What is the accumulated substance in lead poisoning
Protoporphyrin (blood)
SLE - CLL - alpha methyldopa
Dec synthesis of factors 1972 - protein C/S
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

45. How is beta thal minor dx
TPA - cleavage of fibrin mesh
Birbeck granules
Inc HbA2 on electrophoresis
Bleeding - malnutrition/absorption - inc demand pregs

46. What are the azuraphilic granules in PMNs
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Complications of sickle cell anemia
ADP and Ca
Lysosomes

47. What are the likely exposures of kids and adults for lead poisoning
Uroporphyrinogen decarboxylase
Histaminase and arylsulfatase
Kids= exposure to lead paint - adults = battery - ammunition factory
Blod - tissue - MACS

48. What chromosomal translocation is associated with a better prognosis in All
Mantle cell - older males
Produce antibody - lots of RER and golgi
T(12;21)
2 to 10

49. Where to T cells arise and mature - and migrate to...
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Porphyrias
Protoporphyrin (blood)
Paraprotein spike - monoclonal protein

50. How do platelet disorders present?
Leukemia
Fc
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Glycine and succinyl - coa