Ramzi Mani, Rezvani Alireza, Haghighinejad Hourvash
From the Department of Hematology, Medical Oncology and Stem Cell Transplantation, Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Exp Clin Transplant. 2014 Oct;12(5):490-3.
We describe the successful hematopoietic stem cell transplant of a 22-year-old patient with chronic granulomatous disease at our center. Our case was a relatively old male patient compared with other patients having the diagnosis of chronic granulomatous disease who underwent hematopoietic stem cell transplant. The patient was diagnosed as chronic granulomatous disease at the age of 1 year when he developed repeated bacterial infections since 2 months of age and laboratory test revealed 0% of normal control value on nitroblue tetrazolium test. In December 2011, at 22 years of age, he underwent an allogeneic hematopoietic stem cell transplant from human leukocyte antigen fully matched sibling. Conditioning regimen was busulfan 16 mg/kg and cyclophosphamide 120 mg/kg. He received methotrexate and cyclosporine as prophylaxis for graft-versus-host disease. Engraftment was achieved at day 13. At the time of this writing, the patient is alive and well with no serious complication and without any evidence of graft-versus-host disease or rejection 12 months after stem cell transplant.
我们描述了在我们中心对一名患有慢性肉芽肿病的22岁患者进行造血干细胞移植的成功案例。与其他诊断为慢性肉芽肿病并接受造血干细胞移植的患者相比,我们的病例是一名年龄相对较大的男性患者。该患者在1岁时被诊断为慢性肉芽肿病,自2个月大起就反复出现细菌感染,实验室检查显示硝基蓝四氮唑试验结果为正常对照值的0%。2011年12月,22岁的他接受了来自人类白细胞抗原完全匹配的同胞的异基因造血干细胞移植。预处理方案为白消安16mg/kg和环磷酰胺120mg/kg。他接受了甲氨蝶呤和环孢素以预防移植物抗宿主病。在第13天实现了植入。在撰写本文时,患者存活且状况良好,在干细胞移植12个月后没有严重并发症,也没有任何移植物抗宿主病或排斥反应的迹象。