Treatment

Similar to patients with CMML, the most therapeuti-cally relevant diagnostic tests are karyotype analyses, which should be completed with additional testing for the presence of BCR/ABL fusion gene or gene transcripts by fluorescence in situ hybridization (FISH) and, preferably, by molecular testing using PCR. The BCR/ABL-negative CML/aCMLs are rare and only recently defined diseases. No clinical trial has ever been conducted to assess the effectiveness of any specific treatment. Traditionally, the primary goal of therapeutic interventions has been to control myeloproliferation manifested by leukocytosis, or thrombocytosis, and amelioration of anemia, throm-bocytopenia, and systemic clinical symptoms associated with the disease (i.e., fatigue, weigh loss, night sweats, cough, bone pain, and organomegaly). In asymptomatic patients with moderate leukocytosis without anemia or thrombocytopenia, it may be prudent to observe the patient with appropriate hemato-logic follow-up to assess the rate of disease progression.

Younger patients who may be potential candidates for allogeneic SCT should initiate tissue typing of family members and search for a potential stem cell donor. Exceptions to this "observe-and-wait" approach are the rare patients with documented balanced translocations involving RTKs, who are amenable to treatment with TK inhibitors, such as imatinib mesylate, as discussed in the Section "Chronic Myelomonocytic Leukemia".

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