title: Successful Unrelated Cord Blood Stem Cell Transplantation in an X-linked Chronic Granulomatous Disease Patient with Disseminated BCG-induced Infection.
abstract: A 19-month-old boy with chronic granulomatous disease (CGD) received umbilical cord blood transplantation (UCBT) from an unrelated donor after experiencing a life-threatening disseminated Bacillus Calmette-Guérin infection. After busulfan and cyclophosphamide conditioning, we performed a 5/6-matched UCBT. Engraftment and mixed chimerism was 100% in peripheral blood, and 100% of his neutrophils had normal oxidative burst activity on day 17. The patient is now 3 years old, free from infection, and growing well. To our knowledge, this is the second case of CGD treated with UCBT in Taiwan. His successful outcome illustrates that UCBT in a patient with CGD should be considered early if a human leukocyte antigen-matched donor is not available or the patient has just recovered from a severe infection.
authors.affiliation: Department of Pediatrics, Changhua Christian Children's Hospital, Changhua, Taiwan, ROC; School of Medicine, Chung Shan Medical University, Taichung, Taiwan, ROC. Electronic address: 124140@cch.org.tw.
authors.forename: Chao-Jen
authors.initials: CJ
authors.lastname: Lin
authors.affiliation: Department of Pediatrics, Changhua Christian Children's Hospital, Changhua, Taiwan, ROC.
authors.forename: Shih-Chung
authors.initials: SC
authors.lastname: Wang
authors.affiliation: Graduate Institute of Clinical Medical Science, Chang-Gung University, Tao-Yuan, Taiwan, ROC.
authors.forename: Cheng-Lung
authors.initials: CL
authors.lastname: Ku
authors.affiliation: Department of Pediatrics, Changhua Christian Children's Hospital, Changhua, Taiwan, ROC.
authors.forename: Jun-Kai
authors.initials: JK
authors.lastname: Kao
authors.affiliation: Center for Medical Genetics, Changhua Christian Children's Hospital, Changhua, Taiwan, ROC.
authors.forename: Ming
authors.initials: M
authors.lastname: Chen
authors.affiliation: Vascular and Genomic Center, Changhua Christian Children's Hospital, Changhua, Taiwan, ROC.
authors.forename: Chin-San
authors.initials: CS
authors.lastname: Liu
mesh_terms: D036101:Cord Blood Stem Cell Transplantation; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000628:therapy*; D006801:Humans; D007223:Infant; D008297:Male; D009163:Mycobacterium bovis; D013624:Taiwan; D014376:Tuberculosis / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*
keywords: chronic granulomatous disease (CGD); disseminated Bacillus Calmette–Guérin (BCG) infection; umbilical cord blood transplantation (UCBT)