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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 the philadelphia chromosome
Inc large vWF multimers - inc platelet aggregation and thrombosis
Pyruvate kinase def - extravascular
T(9;22) bcr abl
Aspirin

2. What is the receptor for fibrinogen?
GpIIb/IIIa
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Intravascular hemolysis
Extravascular

3. What is the defect in beta thal?
Inactivates it
Idiopathic thrombocytopenia (ITP)
Dec synthesis of factors 1972 - protein C/S
Splicing sites and promotor sequences

4. What is the pattern of involvement and spread for nonHod lympho
Deficiency in factor VIII
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Fibrinogen bind GpIIb/IIIa and links platelts

5. What is the coombs test results in AIHA?
CLL (SLL without the peripheral lymphocytosis
Pos
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
SLE - CLL - alpha methyldopa

6. What are the anti aggregation factors?
Collagen - BM - activated platelets
Parvovirus
PGI2 - NO inc blood flow - dec platelet aggregation
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

7. Blood type A
A antigen and B antibodies
Uroporphyrinogen decarboxylase
Gp1b
AB - no antibodies

8. What do auer rods stain with
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Blood
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Peroxidase

9. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Imatinib - anti bcr abl
Parvovirus
CD15 and CD30 pos
Folate def

10. What is the therapy for the M3 variant?
Inc UCB
All trans retinoic acid
T(12;21)
Blasts > 5%

11. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Diffuse Large b cell lymphoma
Factor V Leiden
Bite cells and Heinz bodies
Lymphoma

12. What are some causes of cold agglutinin anemia
HIV or immunosupression
HbSS
TXA2 - dec blood flow - inc platelet aggregation
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

13. normal RBCs added to patient's serum
Indirect coombs - agglutinate if serum anti RBC surface Ig
Beta thal
Nodular sclerosing
Beta 4

14. Which factor does vWF carry/protect
Bone marrow infiltration - myelofibrosis
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
VIII
Liver disease - abetalipoproteinemia - acntho = spiny

15. Which pathway and factors are tested in the PTT test
Imatinib - anti bcr abl
Intrinsic - all factors except - VII - XIII
Degrades fibrin mesh and converts C3 to C3a
B12 in fatty acid pathways leads to subacute combined degeneration

16. Bite cell
IgG - Warm is GREAT
CML
G6PD
Inducers of primary antibody response

17. What is the result of thrombocytopenia or platelet dysfunction?
Petechiae
Sickle cell anemia
Hairy cell leukemia
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

18. Is G6PD intravascular or extravascular
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Dec
T(9;22) bcr abl
Intravascular

19. Adults present with cutaneous patches/nodules - indolent CD4+
40 to 75
Histaminase and arylsulfatase
TXA2 - dec blood flow - inc platelet aggregation
Mycosis fundgoides/Sezary syndrome

20. What are the presenting symptoms of porphyria cutanea tarda
Plummer - vinson syndrome
Blistering cutaneous photosens - most common porphyria
Beta chain underproduced - asymptomatic - heterozygote
40 to 75

21. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Leukemoid reaction
CML

22. Spherocyte
Beta thal
Exposed collagen upon endothelial damage
VIII
Hereditary spherocytosis - autoimmune hemolysis

23. inc LDH - jaundice
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Inappropriate absolute with inc RBCs and EPO
Adult T cell lymphoma - presents with cutaneous lesions
Extravascular hemolysis

24. What do labs show in DIC?
Nodular sclerosing
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Bernard soulier
Schistocytes - helmet cells

25. Deficiency in GpIb
Bernard soulier
Bleeding - malnutrition/absorption - inc demand pregs
Mycosis fundgoides/Sezary syndrome
Nodular sclerosing

26. What is the life span of a normal RBC
All trans retinoic acid
T(12;21)
120 days
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

27. What condition can result from treating AML M3 from the release of the Auer rods
Bimodal - men>women - except for nodular sclerosing type
DIC
Multiple myeloma
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

28. What are the etiologies of folate def
2 to 10
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
CLL (SLL without the peripheral lymphocytosis
Inappropriate absolute with inc RBCs and EPO

29. Which immunoglobulin is involved in warm agglutination?
ADP and Ca
Contiguous
SLE - CLL - alpha methyldopa
IgG - Warm is GREAT

30. What chromosomal translocation is associated with a better prognosis in All
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
T(12;21)
Exposed collagen upon endothelial damage
Bone marrow infiltration - myelofibrosis

31. What is HbH
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Fibrinogen
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Beta 4

32. What do platelets release necessary for coagulation cascaed?
Malaria - Babesia
ADP and Ca
Ferrocheletase and ALA dehydrogenase
Howell Jolly bodies

33. What is monoclonal expansion without symptoms associated with multiple myeloma?
Blistering cutaneous photosens - most common porphyria
Protoporphyrin (blood)
MGUS - monoclonal gammopathy of undetermined significance
TRAP (tartrate resistant acid phosphatase

34. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Varying sizes
Hereditary spherocytosis
Blod - tissue - MACS
Down - down - up

35. Fibrotic obliteration of bone marow with teardrop cells

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36. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Essential thrombocytosis
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
G6PD
Varying sizes

37. What activates the fibrinolytic pathway?
Schistocytes and inc LDH
IgG - Warm is GREAT
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Induces differentiation of myeloblasts

38. What does 'Neutrophils Like Making Everything Better' stand for?
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Burkitt lymphoma
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
CD15 and CD30 pos

39. Defect in platelet to platelet adhesion - receptor - disorder - platelet count and bleeding time

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40. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
ADP and Ca
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
All
Idiopathic thrombocytopenia (ITP)

41. What is the tx for aplastic anemia
Up - down - up
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Varying shapes
Ewing sarcoma

42. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Varying shapes
Her next Rh+ fetus
Intravascular hemolysis
Adult T cell lymphoma - presents with cutaneous lesions

43. What activates protein C and What does activated protein C do?
Yes - via MHC II
CML
Protein S - cleaves and invactivates Va and VIIIa
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

44. What are the azuraphilic granules in PMNs
DIC - TTP/HUS - traumatic hemolysis
CML to AML or All
Protoporphyrin (blood)
Lysosomes

45. What is the difference of presentation of Burkitt in Africa vs the United States
No lytic bone lesions in WM
Africa = Jaw lesion - US = pelvis or abdomen
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

46. What are some causes of warm agglutinin autoimmune hemolytic anemia?
IFN gama
Inc HbF and dec HbS
Sideroblastic anemia
SLE - CLL - alpha methyldopa

47. Bone pain plus anemia
Hb Barts - gamma4 in defect in all 4 alpha genes
Multiple myeloma
Fibrinogen
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

48. How do platelet disorders present?
Lead - and EtOH
Sickle cell anemia
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

49. Blood type B
Epoxide reductase - warfarin inhibits
Kids= exposure to lead paint - adults = battery - ammunition factory
B antigena and A antibodes
Her next Rh+ fetus

50. What is hemophiliia A
Anti Rh
Deficiency in factor VIII
Acts to stabilize platelet plug
Round densly staining nucleus with a small amount of pale cytoplasm