A Case of Macrophage Activation Syndrome Developing in a Patient With Chronic Granulomatous Disease-associated Colitis
A Case of Macrophage Activation Syndrome Developing in a Patient With Chronic Granulomatous Disease-associated Colitis
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DOI：
10.1097/mph.0b013e31828e5dae
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发表时间：
2014-04-01
影响因子：
1.2
通讯作者：
Onodera, Masafumi
中科院分区：
文献类型：
--
作者：
Akagi, Kazuko;Kawai, Toshinao;Onodera, Masafumi
尽管某些慢性肉芽肿病 (CGD) 患者会出现巨噬细胞激活综合征 (MAS)，但所有报告的病例均与病原微生物感染有关。我们报告了一名患有 CGD 相关结肠炎的 2 岁男孩，他患有 MAS，但没有任何微生物感染的临床症状。给予甲泼尼龙冲击治疗1个疗程，临床症状好转；然而，即使使用免疫抑制药物，结肠炎也难以控制，最终在MAS发病1年后需要进行造血干细胞移植。患有 CGD 结肠炎的患者可能会出现 MAS，与微生物感染无关。
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.