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。

Pub Date : 2024-02-01 Epub Date: 2023-07-27 DOI:10.5546/aap.2023-10061.eng
Selene Pury, Milagros López Orozco, Gabriela Pichichero, Laura V Sasia, Daniela Morell, María S Álvarez, Ana L Basquiera, María E Mas, Karina Salvucci
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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。
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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.

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