Leucemia Mielóide Crônica em pediatria: perspectivas atuais

Abstract

Chronic myeloid leukemia (CML) is a rare event in childhood, comprising of less than 5% of all leukemia cases in this age group. CML is characterized by the presence of a specific molecular marker, the Ph+ chromosome, which is responsible for almost all etiopathogenesis, hence, it has clinical and course characteristics that do not differ from the adult population. In pediatrics the therapeutic approach is based mainly on the experience obtained with adult protocols. With bone marrow transplantation (BMT) being the only cure option, this procedure is more effective in patients with compatible related donors and performed during the initial chronic phase of the disease. The great anti-leukemic efficacy seen with imatinib mesylate was responsible for the approval of this drug in pediatric use for intolerant or refractory -interferon treated patients or relapsed patients after BMT. Currently, its use in pediatric patients with recently diagnosed CML, who have a compatible donor, has become a great dilemma. There is no agreement yet on which is the best way to use this drug or even whether it will ever replace BMT. Further studies with longer follow-up periods are still needed.