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Atypical Chronic Myeloid Leukemia
Treatment
Hydroxyurea is the standard therapy for patients with myelodysplastic/myeloproliferative disease, combined with supportive care to manage symptoms.1 Hematopoietic growth factors, particularly erythropoietin, may be beneficial for some patients. No other treatment except allogeneic stem cell transplantation has been demonstrated to improve the natural history of these diseases, although relapse and graft-versus-host disease are not uncommon occurrences.1,2
Course
Clinically, aCML has been shown to be very aggressive, with median survival times ranging from 11 to 18 months.3 Thrombocytopenia and marked anemia are indicative of a poor prognosis.4
Emerging Therapies for the Treatment of Myelodysplastic / Myeloproliferative Diseases
Several therapeutic approaches that utilize a molecular-targeted approach to the treatment of myelodysplastic/myeloproliferative diseases are currently under investigation.
One such approach involves use of tyrosine kinase inhibitors that target the fusion protein that transforms eosinophilic cells or their stem cells in patients with FIP1L1-PDGFRA-positive disease. This approach shows considerable promise in the management of patients with these frequently fatal disorders. Clinical trials are underway.
Clinical Trials
For U.S. Residents Only
References:
- Cortes J. CMML: a biologically distinct myeloproliferative disease. Curr Hematol Rep. 2003;2:202-208.
- Magnusson MK, Meade KE, Nakamura R, Barrett J, Dunbar CE. Activity of STI571 in chronic myelomonocytic leukemia with a platelet-derived growth factor beta receptor fusion oncogene. Blood. 2002;100:1088-1091.
- Hernandez JM, del Canizo MC, Cuneo A, et al. Clinical, hematological and cytogenetic characteristics of atypical chronic myeloid leukemia. Ann Oncol. 2000;11:441-444.
- National Cancer Institute. Myelodysplastic/Myeloproliferative Diseases (PDQ®): Treatment. Health Professional Version. Available at: http://www.cancer.gov/cancertopics/pdq/treatment/mds-mpd/healthprofessional/allpages. Accessed April 24, 2006.
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