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Chronic Myeloid Leukemia (CML) (a myeloproliferative disorder)…
Chronic Myeloid Leukemia (CML) (a myeloproliferative disorder)
Cells
Mature myeloid cells-- esp. granulocytes and precursors:
Basophil proliferation
t(9;22)
Philadelphia chr. -- generates
BCR-ABL protein
(increased TK activity to drive proliferation)
Clinical Feat.
Splenomegaly
-- accelerated phase (transformation to Acute Leukemia will follow shortly)
Can
transform to AML (2/3 cases) or ALL (1/3 cases)
--
mutation in pluripotent stem cell
Distinguishing Feat.
(-) Leukocyte alkaline phosphatase
(LAP) stain
Increased
basophils
t(9;22)
Compared to Leukemoid Rxn (reactive neutrophilic leukocytosis):
Treatment
Imatinib
-- blocks TK activity
Phases
Blast Crisis
Myeloid
t(9;22)
Lymphoid
Accelerated
6-19% blasts in BM
Chronic
Thrombocytosis
<10% blasts in BM
Leukocytosis