Selene Pury, Milagros López Orozco, Gabriela Pichichero, Laura V Sasia, Daniela Morell, María S Álvarez, Ana L Basquiera, María E Mas, Karina Salvucci
{"title":"一名患有 Wiskott-Aldrich 综合征的儿童患者的单倍体骨髓移植。病例报告。","authors":"Selene Pury, Milagros López Orozco, Gabriela Pichichero, Laura V Sasia, Daniela Morell, María S Álvarez, Ana L Basquiera, María E Mas, Karina Salvucci","doi":"10.5546/aap.2023-10061.eng","DOIUrl":null,"url":null,"abstract":"<p><p>Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder caused by mutations in the gene that encodes the Wiskott-Aldrich syndrome protein (WASp). Here, we report the clinical case of an 18-month-old boy diagnosed with Wiskott-Aldrich syndrome, who did not have an HLA-matched related or unrelated donor and was treated successfully with a hematopoietic stem cell transplant (HSCT) from a haploidentical family donor. Graft-versus-host disease (GvHD) prophylaxis included post-transplant cyclophosphamide (PT-Cy). At day +30, the peripheral blood-nucleated cell chimerism was 100% and the WAS protein had a normal expression. Currently, at month 32 post-transplant, the patient has hematological and immune reconstitution and complete donor chimerism without evidence of GvHD. HSCT with PT-Cy was a feasible and safe option for this patient with WAS, in which an HLA matched donor was not available.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":" ","pages":"e202310061"},"PeriodicalIF":0.5000,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Haploidentical bone marrow transplantation in a pediatric patient with Wiskott-Aldrich syndrome. A case report.\",\"authors\":\"Selene Pury, Milagros López Orozco, Gabriela Pichichero, Laura V Sasia, Daniela Morell, María S Álvarez, Ana L Basquiera, María E Mas, Karina Salvucci\",\"doi\":\"10.5546/aap.2023-10061.eng\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder caused by mutations in the gene that encodes the Wiskott-Aldrich syndrome protein (WASp). Here, we report the clinical case of an 18-month-old boy diagnosed with Wiskott-Aldrich syndrome, who did not have an HLA-matched related or unrelated donor and was treated successfully with a hematopoietic stem cell transplant (HSCT) from a haploidentical family donor. Graft-versus-host disease (GvHD) prophylaxis included post-transplant cyclophosphamide (PT-Cy). At day +30, the peripheral blood-nucleated cell chimerism was 100% and the WAS protein had a normal expression. Currently, at month 32 post-transplant, the patient has hematological and immune reconstitution and complete donor chimerism without evidence of GvHD. HSCT with PT-Cy was a feasible and safe option for this patient with WAS, in which an HLA matched donor was not available.</p>\",\"PeriodicalId\":8338,\"journal\":{\"name\":\"Archivos argentinos de pediatria\",\"volume\":\" \",\"pages\":\"e202310061\"},\"PeriodicalIF\":0.5000,\"publicationDate\":\"2024-02-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Archivos argentinos de pediatria\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.5546/aap.2023-10061.eng\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2023/7/27 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"PEDIATRICS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Archivos argentinos de pediatria","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.5546/aap.2023-10061.eng","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2023/7/27 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"PEDIATRICS","Score":null,"Total":0}
引用次数: 0
摘要
威斯科特-阿尔德里奇综合征(WAS)是一种 X 连锁遗传病,由编码威斯科特-阿尔德里奇综合征蛋白(WASp)的基因突变引起。在此,我们报告了一个临床病例,一名18个月大的男童被诊断患有威斯科特-阿尔德里奇综合征,他没有HLA匹配的亲缘或非亲缘供体,但接受了来自单倍体家族供体的造血干细胞移植(HSCT),并取得了成功。移植抗宿主病(GvHD)的预防措施包括移植后环磷酰胺(PT-Cy)。移植后第 30 天,外周血有核细胞嵌合率为 100%,WAS 蛋白表达正常。目前,在移植后第 32 个月,患者的血液和免疫功能均已重建,供体嵌合完全,没有出现 GvHD 的迹象。对于这名无法获得 HLA 匹配供体的 WAS 患者来说,使用 PT-Cy 进行造血干细胞移植是一种可行且安全的选择。
Haploidentical bone marrow transplantation in a pediatric patient with Wiskott-Aldrich syndrome. A case report.
Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder caused by mutations in the gene that encodes the Wiskott-Aldrich syndrome protein (WASp). Here, we report the clinical case of an 18-month-old boy diagnosed with Wiskott-Aldrich syndrome, who did not have an HLA-matched related or unrelated donor and was treated successfully with a hematopoietic stem cell transplant (HSCT) from a haploidentical family donor. Graft-versus-host disease (GvHD) prophylaxis included post-transplant cyclophosphamide (PT-Cy). At day +30, the peripheral blood-nucleated cell chimerism was 100% and the WAS protein had a normal expression. Currently, at month 32 post-transplant, the patient has hematological and immune reconstitution and complete donor chimerism without evidence of GvHD. HSCT with PT-Cy was a feasible and safe option for this patient with WAS, in which an HLA matched donor was not available.
期刊介绍:
Archivos Argentinos de Pediatría is the official publication of the Sociedad Argentina de Pediatría (SAP) and has been published without interruption since 1930. Its publication is bimonthly.
Archivos Argentinos de Pediatría publishes articles related to perinatal, child and adolescent health and other relevant disciplines for the medical profession.