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Myeloproliferative disorders MPD , also called Myeloproliferative…

- - - - 3 distinctive phase
        
        Accelerated phase or blast crisis: resembling acute leukemia, very poor prognosis. increase in blast percentage in the BM/PB
        
        Blast crisis:poor prognosis. over 20% blast
        
        Chronic phase: proliferation of myeloid cells with full range of maturation. increase Wbc but blast percentage less 5%.
      - stem cell stage proliferation of myeloid elements at all stages of differentiation. and associated with Philadelphia chromosome that is t(9;22) and/or BCR/ABL fusion gene. (d/t 22gene changed and can create BCR)
      - granulocytes: neutrophils and basophils
      - 90% CML is p210 BCR- ABL. that can increase proliferative capacity, slight delay in maturation, lack of responsiveness to growth regulators and prevent apotosis of malignant Cml.
      - cytogenetic abnormality: is positive of translocation of Philadelphia t(9;22) result in fusion gene BCR/ABL
      - Lab: In BM hypercellularity with granular proliferation. In PB: leukocytosis > 50x 10 <9>/L. with immature granulocytes all stages. Basophilia, that's a prominent feature. RBC NC/NC (Normocytic/Normochromic)
      - LAP: Leukocyte alkaline phosphatase. is enzyme of leukocyte. but malignant leukocytes not function, so markedly decrease in CML (**this is correct)
      - decrease LAP. CD 13,14,15,33. megakecytes CD 41,61, lymphoid mark 3, 7.
      - 90% breakpoint is P210. Use Gleevec (imatinib mesylate or STI 571). is good reaction. If resist, the new drugs; Dasatinib