RESUMEN

We report four cases of Noonan syndrome associated with chronic myelomonocytic leukemia in childhood. These children shared some hematologic features: thrombocytopenia, splenomegaly in the first months of life, occurrence of chronic myelomonocytic leukemia without abnormalities of the initial bone marrow karyotype, and, in three cases, improvement of the hematologic disease. A common pathophysiologic process in such patients is suggested.

Asunto(s)

Leucemia Mielomonocítica Crónica/complicaciones , Síndrome de Noonan/complicaciones , Médula Ósea/química , Femenino , Humanos , Lactante , Recién Nacido , Cariotipificación , Leucemia Mielomonocítica Crónica/diagnóstico , Masculino , Síndrome de Noonan/diagnóstico , Estudios Retrospectivos , Esplenomegalia/tratamiento farmacológico , Esplenomegalia/etiología , Trombocitopenia/diagnóstico , Trombocitopenia/etiología