Sastry Jairam, Kakakios Alyson, Tugwell Heidi, Shaw Peter J
Oncology Unit, The Children's Hospital at Westmead, University of Sydney, Sydney, New South Wales, Australia.
Pediatr Blood Cancer. 2006 Sep;47(3):327-9. doi: 10.1002/pbc.20865.
Chronic granulomatous disease (CGD) is a rare disorder characterized by recurrent infections, often resulting in impaired quality of life and death. Allogeneic BMT provides a definitive cure for CGD, but carries a significant risk of mortality and morbidity. The risk is higher for those who have invasive fungal infection prior to transplant. Reduced intensity conditioning (RIC) is associated with less toxicity from the conditioning agents and may provide an alternative option for all non-malignant diseases. We report a case of successful allogeneic BMT after RIC for a case of X-linked CGD complicated by severe invasive aspergillosis (IA).
慢性肉芽肿病（CGD）是一种罕见的疾病，其特征为反复感染，常导致生活质量受损甚至死亡。异基因骨髓移植（BMT）可为CGD提供根治方法，但存在显著的死亡和发病风险。对于移植前发生侵袭性真菌感染的患者，该风险更高。减低预处理强度（RIC）与预处理药物的毒性较低相关，可能为所有非恶性疾病提供另一种选择。我们报告了1例接受RIC后成功进行异基因BMT治疗的X连锁CGD合并严重侵袭性曲霉病（IA）的病例。
