Umeda Katsutsugu, Adachi Souichi, Horikoshi Yasuo, Imai Kohsuke, Terui Kiminori, Endo Mikiya, Mitsui Tetsuo, Kato Keisuke, Koh Katsuyoshi, Kajiwara Ryosuke, Ito Rieko, Otsuka Yoshitoshi, Inoue Masami, Ishii Eiichi, Yabe Hiromasa
Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Human Health Sciences, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Pediatr Transplant. 2016 Mar;20(2):271-5. doi: 10.1111/petr.12626. Epub 2015 Oct 29.
The clinical outcome of allogeneic HSCT was retrospectively analyzed in eight patients with CHS. In total, six of these eight patients are alive. Four of five patients transplanted with MAC achieved prompt engraftment, and three of the four patients, including two patients with AP before transplant, are alive without disease. In contrast, three patients transplanted with RIC without active AP disease achieved prompt engraftment and survive long term. RIC-HSCT might be an alternative treatment for CHS similar to other types of HLH, at least for patients without active AP disease.
对8例慢性肉芽肿病（CHS）患者接受异基因造血干细胞移植（HSCT）的临床结果进行了回顾性分析。这8例患者中共有6例存活。接受清髓性预处理（MAC）移植的5例患者中有4例迅速植入，4例患者中的3例存活且无疾病，其中包括2例移植前有加速期（AP）的患者。相比之下，3例接受减低强度预处理（RIC）且无活动性AP疾病的患者迅速植入并长期存活。RIC-HSCT可能是CHS的一种替代治疗方法，类似于其他类型的噬血细胞性淋巴组织细胞增生症（HLH），至少对于无活动性AP疾病的患者是这样。
