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babybless88
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2010-04-26 16:23:48
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WBC anomalies
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WBC anomalies
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  1. What is the def. of WBC anomalies?
    congenital or hereditary condition where the WBC's deviate from the normal standard
  2. what is the Etiology of Inherited (true) Pelger-huet?
    • -autosomal dominant
    • -abnormal maturation of nucleus
    • -appear to function normally
  3. What are the laboratory features of an Inherited (true) Pelger-huet?
    • -decreased segmentation of nucleus
    • -coarse and condense chromatin
  4. this cell maybe drug induced, is secondary to leukemia(CmL) and is 10% normal 3 lobed nucleus
    Pseudo-Pelger-Huet
  5. Pelger-Huet
  6. this cell is shift to the left, metamyelocytes and bands are present and is a response to severe infection
    Pelger-Huet
  7. chediak-higashi
  8. this is a defect in the lysomes and it leads to delayed degranulation(stained blue-purple granules)
    chediak-higashi
  9. this cell is autosomal dominant
    May-Hegglin
  10. What are the laboratory features of a may-hegglin
    • -dohle bodies(blue straining area made up of rough plasma eticular & RNA)
    • -thrombocytopenia
    • -giant, bizarre platelets
  11. this cell is rare sex-linked recessive, fatal due to infetion, and defect in WBC; unable to kill catalase positive organism
    CGD
  12. What are the laboratory features of CGD
    • -normal WBC morphology
    • -WBC increases during infection
    • -granulomas in many organs
    • *granular tumor growth
    • *sites of chronic infection
  13. this disease is the deficiency of enzyme beta-glucocerebroside
    Gausher's diesease
  14. what are the laboratory findings of Gaucher's disease
    • -leukopenia
    • -thrombocytopenia
    • -hepatosplenomegaly
    • -in bone marrow looks like unfolded crumpled paper
    • -increased serum acid phosphatase
  15. what is the etiology of tay-sachs disease?
    • -autosomal recessive
    • -deficiency oof hexaminosidase
    • -seen in mostly jews
  16. what is hexaminosidase?
    accumulation of sphingolipids in lymphocytes
  17. this disease death will occur within a few a years, and is a major site of pathology is central nervous system
    Tay-sachs disease
  18. what is the etiology of a neiman-pick?
    • -aka Sea-blue histiocytes
    • -due to defiency in sphigomyelinase
    • (leads to accumulation of sphigomyelin in histocytes)
  19. this the deficiency in enzymes that break down mucopolysaccharide
    Hunter's & Hurler's
  20. this cell is caused by EBV(Epstein Barr Virus)-infects the B-lymphocytes, affects all races mostly children and young adults, enter the body orally
    infectious monocucleosis
  21. What are the symptoms of infectious monocucleosis?
    • -general malaise
    • -enlarge lymph nodes
    • -splenomegaly
    • -hepatomegaly
    • -benign illness
  22. atypical lymphocyte
  23. what is the etiology of Leukemoid reaction?
    • -neutrophilic response to infection
    • -accelerated release of neutrophil
    • -mimics early stage of leukemia
    • (hence leukemoid reaction)
  24. this has dark blue-black granules seen in severe infection (toxic granulation)
    leukemoid reaction
  25. dohle bodies
  26. explain the Leukomoid reaction vs. CML
    • WBC count---10-100---30-500
    • Eosinophils---absent---present
    • Basophils---absent---present
    • Philadelphia chromosome---absent---present
    • LAP---inceased---decreased
  27. this is the malignat disease of hematopoietic tissue
    Leukemias
  28. this is normal bone marrow elements replaced by neoplastic cells, bone marrow is always involved, and infiltates spleen, liver, lymph nodes
    leukemias
  29. explain the acute vs. chronic
    • ------------acute------chronic
    • age:------all ages----adults
    • onset:----sudden-----insidous
    • cells:-----immature(blast)----mature
    • anemia--mild to severe--mild
  30. this will become mild-severe when acute and mild when chronic
    Thrombocytopenia
  31. what are the laboratory features of acute leukemia?
    • -WBC count:variable
    • -WBC differential: present and usually predominate blast
    • -normocytic, normochromic anemia if liver and spleen not affected
  32. what is the purpose of FAB classification
    to attain better discrimination in therapy and prognosis
  33. what are the basis of FAB classification
    • -WBC morphology
    • -cytochemical staining
    • -immunologic marker studies
    • -cytogenetics
  34. explain the basis of FaB classification
    • wbc morphology:
    • -----------AML Blast vs ALL Blast
    • -size-----large-----------small
    • -cytoplasm:moderate--scant
    • -chromatin:fine,lace---dense
  35. this is prominent AML blast usually >2
    nucleoli
  36. this indistinct ALL Blast usually 2 or less
    nucleoli
  37. this is present in 50-60% in AML Blast
    Auer Rods
  38. this is never present in ALL Blast
    Auer Rods
  39. this is the presence or absence of enzymes or lipids differentiate hematopoietic cells, and aids in diagnosis and classification of leukemia
    cytochemical staining, Basis of Fab Classification
  40. Leukemic cells are ___% primitive leukemic blast
    100%
  41. What are the characteristics of M2-AML
    • -resembles M1 except smear shows maturation to or beyond promyeloctes
    • *M1 and M2 combined are most common type of AML
    • -auer rods are present
  42. M2 AML
  43. In M3-APL leukemic cells are promyelocytes that has what?
    • -kidney shaped or bilobed nucleus
    • -moderate cytoplasm
    • -heavy granulation
    • -auer rods common & in multiple
    • -negative chemical test
  44. in M3-APL coagulation abnormalities include what?
    • -High incidence of DIC
    • -prolonged PT, PTT
    • - elevated FDP
    • -decreased Factors I and V
    • -thrombocytopenia
  45. M3-APL
  46. Leukemic cells are called what?
    myelomonocytic cells
  47. M4-AMML are characterized by both what?
    monocytic and granulocytic differentiation
  48. this leukemia is positive for periodic acid-Schiff (PAS) stain
    M6-AEL
  49. What is the size of L2-ALL?
    • -larger than L1
    • -heterogenous
  50. What does the nucleus on a L2-ALL look like?
    • -irregular in shape
    • -clefting & indentation common
    • -heterogenous chromatin pattern
    • -one or more nucleoli
  51. What does the cytoplasm look like on L2-ALL?
    • -variable, often moderately abundant
    • -variable cytoplasmic vacuolization
  52. B-cell ALL is also called what?
    Burkitt's leukemia or lymphoma
  53. this is asymmetrical enlargement of lymph nodes
    lymphoma
  54. B-cell ALL accounts for __-__ % of ALL cases
    2-5%
  55. Name the types of chronic Leukemia
    • -chronic lymphocytic leukemia
    • -chronic myelogenous leukemia
    • -chronic myeloid Leukemia
    • -chronic myelocytic leukemia
    • -chronic granulocytic leukemia
  56. this has no specific etiologic agent, most common type of leukemia in adults, and twice more common in men
    CLL
  57. this is 90-95% of cases associated w/ Philadelphia chromosome(conjoing of chromosome 9 and 22, designatedt(9;22)
    CML
  58. name the symptoms of CML
    • -weight loss-stress sign
    • -enlarged spleen
    • -fever
    • -night sweats
    • -malaise
  59. give the definition of Plasma cell Dirorders
    a diverse group of diseases characterized by proliferation of a single clone of cells producing a monoclonal immunoglobin or immunoglobulin fragments
  60. name the etiology of multiple myeloma
    • -radiation
    • -chemical agents
    • *mostly agricultural
    • -certain medical conditions
    • -not normally consider a myeloproliferative disorder
  61. the some signs and symptoms of mutiple myeloma
    • -bone lesions
    • *bone pain in 2/3 of cases
    • -anemia
    • *fatigue SOB
    • -GI problems
    • *constipation, abdominal pain
  62. this involves IgG or IgA, marked rouleux, elevated ESR, and presence of flames cells in BM
    Multiple Myeloma
  63. this is over production of IgM, leads to hyperviscosity syndrome, seen in elderly 60-70 years old and equally in both sexes
    Waldenstrom's Macroglobulinemia
  64. What are some laboratory features of Waldenstrom's Macroglobulinemia?
    • -predominance of prolymphocytes in bone marrow
    • -marked rouleaux
  65. this present in neutrophil, and hydrolyzes napthol AS-BI phosphate
    LAP
  66. What is the principle of MPS?
    • -H2O in the presence of peroxidase reacts w/ 3-amino-9-ethylcarbazole
    • -blue-black precipitate forms
  67. List the purpose of MPS(mylo cells)
    • -differentiate AML or AMol leukemia from ALL
    • *positive for AML and AMol
    • *negative for ALL
  68. name the principle of SBB
    • -lipids are present in granules of Granulocytes and Monocytes
    • -SBB is souluble in lipids
    • -SBB penetrates granules
    • *granules stains black
  69. What are the principle of NSE
    • -sodium FLouride inhibits NSE activity in monocytes and macrophages
    • -sodium fluoride does not inhibit NSE in other cells
  70. this is absent in monocytes
    SE(specific esterase)

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