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J Pediatr Hematol Oncol. 2014 Apr;36(3):e169-72. doi: 10.1097/MPH.0b013e31828e5dae.

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

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Departments of *Interdisciplinary Medicine †Human Genetics ‡Division of Gastroenterology, National Center for Child Health and Development §Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan.


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.

[Indexed for MEDLINE]

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