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 unrela...

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Veröffentlicht in:Archivos argentinos de pediatría 2024-02, Vol.122 (1), p.e202310061-e202310061
Hauptverfasser: Pury, Selene, López Orozco, Milagros, Pichichero, Gabriela, Sasia, Laura V, Morell, Daniela, Álvarez, María S, Basquiera, Ana L, Mas, María E, Salvucci, Karina
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Sprache:eng
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Zusammenfassung: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.
ISSN:0325-0075
1668-3501
DOI:10.5546/aap.2023-10061.eng