Shigemura Tomonari, Nakazawa Yozo, Hirabayashi Koichi, Kobayashi Norimoto, Sakashita Kazuo, Agematsu Kazunaga, Koike Kenichi
Department of Pediatrics, Shinshu University School of Medicine, 3-1-1, Asahi, Matsumoto, 390-8621, Japan.
Department of Infection and Host Defense, Shinshu University, Graduate School of Medicine, Matsumoto, Japan.
J Clin Immunol. 2015 Jan;35(1):84-6. doi: 10.1007/s10875-014-0113-5. Epub 2014 Nov 4.
Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by defects of nicotinamide adenine dinucleotide phosphate oxidase. Catalase-positive bacteria and fungi are phagocytosed, but persist within phagocytes, resulting in granulomatous inflammation. Although allogeneic hematopoietic stem cell transplantation (HSCT) is a curative treatment for CGD, HSCT sometimes leads to fatal outcomes related to the exacerbation of persistent infectious or post-infectious inflammatory diseases, particularly in adolescent and young adult patients with a history of recurrent infections and/or multiple granulomas in organs. Here, we present the case of a young adult with X-linked CGD in whom multiple lesions were found in lungs and lymph nodes on both computed tomography and positron emission tomography (PET) scans before allogeneic HSCT, but all the lesions disappeared only on PET scan 5 months after HSCT. Monitoring the activity of multiple pre-existing lesions with PET scan may be beneficial to adolescent and young adult CGD-patients undergoing allogeneic HSCT.
慢性肉芽肿病(CGD)是一种由烟酰胺腺嘌呤二核苷酸磷酸氧化酶缺陷引起的原发性免疫缺陷病。过氧化氢酶阳性细菌和真菌被吞噬,但会在吞噬细胞内持续存在,导致肉芽肿性炎症。尽管异基因造血干细胞移植(HSCT)是治疗CGD的一种治愈性方法,但HSCT有时会导致与持续性感染或感染后炎症性疾病加重相关的致命后果,尤其是在有反复感染史和/或器官中有多个肉芽肿的青少年和年轻成年患者中。在此,我们报告一例年轻成年X连锁CGD患者,在异基因HSCT前,计算机断层扫描和正电子发射断层扫描(PET)均显示其肺部和淋巴结有多个病灶,但HSCT后5个月仅PET扫描显示所有病灶消失。对接受异基因HSCT的青少年和年轻成年CGD患者,用PET扫描监测多个原有病灶的活性可能有益。