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A case of macrophage activation syndrome developing in a patient with chronic granulomatous disease-associated colitis

Journal of Pediatric Hematology/Oncology, ISSN: 1536-3678, Vol: 36, Issue: 3, Page: e169-72
2014
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Article Description

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. © 2014 by Lippincott Williams & Wilkins.

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