RESUMO
The experience with myeloproliferative diseases (MPD) in children of a large pediatric hematology-oncology service during a 20-year period is reviewed. Twenty-seven patients with myeloproliferative diseases were treated, six with the juvenile type of chronic myelogenous leukemia (CML), 10 with the adult type of CML, three with familial MPD, one with unclassifiable MPD, and seven with acute leukemia in whom myelofibrosis was either a prodromal or terminal event. The literature is reviewed with particular emphasis regarding the relationships between the juvenile type of CML and monocytic leukemia, the adult type of CML and acute nonlymphocytic leukemia, and the relationship of myelofibrosis and myeloid metaplasia to the acute leukemias. New therapeutic approaches are needed in this heterogenous but interrelated group of disorders.
Assuntos
Transtornos Mieloproliferativos/diagnóstico , Adolescente , Corticosteroides/uso terapêutico , Bussulfano/uso terapêutico , Transformação Celular Neoplásica , Criança , Pré-Escolar , Cromossomos Humanos 21-22 e Y , Feminino , Hemoglobina Fetal/biossíntese , Humanos , Lactente , Leucemia Eritroblástica Aguda/complicações , Leucemia Mieloide/complicações , Leucemia Mieloide/diagnóstico , Leucemia Mieloide/etiologia , Masculino , Metotrexato/uso terapêutico , Transtornos Mieloproliferativos/tratamento farmacológico , Transtornos Mieloproliferativos/genética , Mielofibrose Primária/complicações , Mielofibrose Primária/diagnóstico , Tioinosina/uso terapêuticoRESUMO
Ten patients with late-stage acute lymphocytic leukemia were treated with L-asparaginase and cytosine arabinoside. Complete remission was achieved in 8 patients including 5 of 5 patients with T-cell leukemia. Major toxicity included anaphylactic reactions in 3 of the 10 patients.
