Janjindamai C.Manuyakorn W.Anurathapan U.Pakakasama S.Wattanasirichaigoon D.Kamchaisatian W.Benjaponpitak S.Hongeng S.Mahidol University2023-06-182023-06-182022-09-01Asian Pacific Journal of Allergy and Immunology Vol.40 No.3 (2022) , 278-2820125877Xhttps://repository.li.mahidol.ac.th/handle/123456789/84921Background: HLA-matched hematopoietic stem cell transplantation (HSCT) is the curative treatment for chronic granulomatous disease (CGD). Objective: To report a case of X-linked CGD with active infection successfully treated by haploidentical HSCT with post-transplant high dose cyclophosphamide (PTCY). Methods: A 5-year-old Thai boy with CGD was undergone for haploidentical HSCT using PTCY with correction of the phagocytic function. He presented with Chromobacterium violaceum liver abscess at the age of 9 months and experienced recurrent perianal abscess and invasive pulmonary aspergillosis even receiving antimicrobial prophylaxis. PTCY was given on day 3 and 4, after CD34+ cells infusion. Results: The peripheral blood-nucleated cell chimerism showed 100% on day 16 and remained 100%. Dihydrorhodamine (DHR) assay on day 108 and day 214 showed normal results. Currently at 22 months post HSCT, he does not receive antibiotic and anti-fungal prophylaxis. Conclusions: Haploidentical HSCT with PTCY could be an effective treatment option for children with CGD.Immunology and MicrobiologyArticleSCOPUS10.12932/ap-290419-05512-s2.0-851363402732228869431677618