A 52-Year-Old Man With Fatigue, Leukocytosis, and Moderate Splenomegaly

Koyamangalath Krishnan, MD

Disclosures

December 11, 2017

Laboratory features also help in the identification of accelerated- and blastic-phase disease. The signature hematopathologic indicator of the blastic phase is a blood or marrow blast percentage greater than 20%.

The following are recommended in the work-up for a patient suspected of having CML[2]:

  • History and physical examination, including spleen size

  • CBC with differential and platelet count

  • Chemistry profile

  • Human leukocyte antigen testing (if allogenic hematopoietic stem cell transplantation is being considered)

  • Bone marrow aspiration and biopsy

    • Percentage of blasts

    • Percentage of basophils

    • Bone marrow cytogenetic analysis

  • FISH studies

  • Quantitative RT-PCR using the international scale on peripheral blood or bone marrow (peripheral blood is preferred because the test needs to be repeated during the course of treatment and offers a baseline)

  • Risk score using either the Sokal index or Hasford score

Two prognostic scoring systems are used: the Sokal index[3] and the Hasford score.[4] Although these scores were tested in the pre-tyrosine kinase inhibitor era, they are still applicable. Both systems include the percentage of circulating blasts, spleen size, platelet count, and age. In addition, the Sokal index includes cytogenetic clonal evolution, whereas the Hasford score includes percentage of eosinophils and basophils instead. The systems identify low-, intermediate-, and high-risk CML on the basis of the above factors.

Some disorders that cause leukocytosis and splenomegaly are Ph-negative and BCR-ABL negative, are not typical CML, and do not respond to tyrosine kinase inhibitors. Atypical CML is such a disorder. A second mimic is chronic neutrophilic leukemia, which is BCR-ABL negative; it needs to be differentiated from neutrophilic CML, which is a rare variant of CML characterized by the presence of a p230 BCR-ABL fusion transcript.

The incidence of CML is 1-2 cases per 100,000 persons, with a slight male preponderance. The median age is 50-60 years. CML has been reported in children aged 10-14 years, which accounts for 2% of childhood leukemia. Etiologic studies have excluded an association between CML and exposure to solvents, pesticides, or alkylating agents. Radiation may be causative (eg, Chernobyl nuclear fallout).[1,5]

Tyrosine kinase inhibitors have radically transformed the treatment landscape and survival in CML.[1,6] Most patients with CML now remain in clinical or cytogenetic remission for 5-7 years or longer. In select patients, allogeneic stem cell transplantation offers a chance of cure.

Available treatment options include the following:

  • Tyrosine kinase inhibitors

  • Allogeneic stem cell transplantation

  • Clinical trial enrollment

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