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Juvenile Myelomonocytic 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
Historically, more than 90% of patients with JMML have died despite the use of chemotherapy. The median survival ranges from approximately 10 months to more than 4 years. The prognosis is relatively better in children younger than 1 year at the time of diagnosis and relatively much worse in children older than 2 at the time of diagnosis.3

Clinical Trials
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References:
  1. Cortes J. CMML: a biologically distinct myeloproliferative disease. Curr Hematol Rep. 2003;2:202-208.
  2. 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.
  3. 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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