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Introduction

Chronic myeloid leukemia (CML) is a myeloproliferative disorder that is characterized by increased production of the granulocytic cell line without loss of the ability to differentiate. CML accounts for 10% to 15% of all leukemias. CML is uncommon before 20 years of age, but the incidence increases with age (mean age is 65 years). CML has three stages: (1) chronic, (2) transformation, and (3) accelerated or blast crisis.

Pathophysiology

CML arises from a mutation in the myeloid stem cells. Normal myeloid cells continue to be produced, but there is a pathologic increase in the production of forms of blast cells. Marrow expands into cavities of the long bones, and cells are formed in the liver and spleen, with resultant painful enlargement problems. A cytogenetic abnormality termed the Philadelphia chromosome is found in 90% to 95% of patients. Patients diagnosed with CML in the chronic phase have few symptoms and complications, with an overall median life expectancy well exceeding 5 years. The transformation phase can be insidious or rapid; it marks the process of evolution (or transformation) to the acute form of leukemia (blast crisis). If the disease transforms to the acute phase (blast crisis), the disease becomes more difficult to treat. Infection and bleeding are rare until the disease transforms to the acute phase.

Clinical Manifestations

Medical Management

Pharmacologic Therapy

  • An oral formulation of a tyrosine kinase inhibitor, imatinib mesylate (Gleevec), is prescribed; this is most useful in the chronic stages.
  • Other tyrosine kinase inhibitors (dasatinib [Sprycel] or nilotinib [Tasigna]) are also approved for primary therapy; each has a slightly (but importantly) different toxicity profile.
  • In those instances where imatinib (at conventional doses) does not elicit a molecular remission, or when that remission is not maintained, other treatment options may be considered.
  • Hematopoietic stem cell transplantation (HSCT) is an additional treatment strategy for patients younger than 65 years. CML can potentially be cured with HSCT in otherwise healthy patients who are younger than 65 years.
  • In the acute form of CML (blast crisis), treatment may resemble induction therapy for acute leukemia, using the same medications as for AML or ALL.
  • Oral chemotherapeutic agents, typically hydroxyurea (Hydrea) or busulfan (Myleran), may be used, as may leukapheresis (for leukocyte count greater than 300,000/mm3). An anthracycline chemotherapeutic agent (e.g., daunomycin [Cerubidine]) may be prescribed for purely palliative approach (rare).

Nursing Management

Nursing management is similar to that for CLL. See “Nursing Process” under “Leukemia” for additional information.

For more information, see Chapter 34 in Hinkle, J. L., & Cheever, K. H. (2018). Brunner and Suddarth's textbook of medical-surgical nursing (14th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.