Chapter 101

Chronic Myeloid Leukemia

Hagop Kantarjian and Jorge Cortes

Summary of Key Points

Incidence

• About 5000 cases of chronic myeloid leukemia (CML) occur per year in the United States; CML represents 15% of all leukemias.

• Median age is 55 to 60 years at diagnosis.

Clinical Findings

• Common findings include fatigue, anemia, abdominal discomfort, splenomegaly, and leukocytosis.

• At diagnosis 40% to 50% of patients are asymptomatic.

• White blood cell count is usually greater than 50 × 10⁹ cells/L, with a left-shifted differential, basophilia, and thrombocytosis.

Differential Diagnosis

• Chronic myelomonocytic leukemia

• Other myeloproliferative disorders

• Leukemoid reactions

Evaluation

• History and physical examination, complete blood cell count with differential and platelet count, and chemistries

• Bone marrow aspiration and biopsy

• Testing for the presence of the Philadelphia (Ph) chromosome by cytogenetic analysis; testing for the presence of the BCR-ABL fusion gene by fluorescent in situ hybridization (FISH) and by quantitative polymerase chain reaction (qPCR) analysis

Therapy

• Hydroxyurea or a BCR-ABL tyrosine kinase inhibitor (TKI; e.g., imatinib mesylate, nilotinib, dasatinib) is given initially to control leukocytosis and thrombocytosis.

• Imatinib induces a complete hematologic and cytogenetic response in most patients; estimated 8- to 10-year survival rate is 85% (93% if only CML-related deaths considered).

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