RESUMEN

Although macrophage activation syndrome (MAS) develops in some patients with chronic granulomatous disease (CGD), all of the reported cases have been associated with pathogenic microbial infections. We report a 2-year-old boy with CGD-associated colitis who suffered from MAS without any clinical signs of a microbial infection. He was treated with 1 course of methylprednisolone pulse therapy and the clinical symptoms improved; however, the colitis was difficult to control even with immunosuppressive drugs, and he eventually required hematopoietic stem cell transplantation 1 year after the onset of MAS. It is likely that MAS develops in patients with CGD colitis independent of microbial infections.

Asunto(s)

Colitis/complicaciones , Enfermedad Granulomatosa Crónica/complicaciones , Trasplante de Células Madre Hematopoyéticas , Síndrome de Activación Macrofágica/etiología , Metilprednisolona/uso terapéutico , Antiinflamatorios/uso terapéutico , Preescolar , Colitis/tratamiento farmacológico , Colitis/patología , Terapia Combinada , Enfermedad Granulomatosa Crónica/tratamiento farmacológico , Enfermedad Granulomatosa Crónica/patología , Humanos , Síndrome de Activación Macrofágica/patología , Síndrome de Activación Macrofágica/terapia , Masculino , Pronóstico