Malignant clonal disorder of hematopoietic stem cells.
At presentation, one third of patients may be asymptomatic; if present, symptoms typically include malaise, fever, weight loss, abdominal discomfort, and night sweats.
Splenomegaly is the most common physical finding; nearly all patients will have elevated WBC count.
Presence of Philadelphia chromosome and /or molecular demonstration of the BCR-ABL transcript confirms diagnosis.
Treatment with a tyrosine kinase inhibitor (e.g., imatinib, dasatinib, or nilotinib), which inhibits the BCR-ABL tyrosine kinase, gives long-term remission without significant adverse events in most patients.