Haploidentical bone marrow transplantation in a pediatric patient with Wiskott-Aldrich syndrome. A case report.
Selene PuryMilagros López OrozcoGabriela PichicheroLaura V SasiaDaniela MorellMaría S ÁlvarezAna Lisa BasquieraMaría E MasKarina SalvucciPublished in: Archivos argentinos de pediatria (2023)
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.
Keyphrases
- case report
- bone marrow
- peripheral blood
- hematopoietic stem cell
- allogeneic hematopoietic stem cell transplantation
- cord blood
- stem cell transplantation
- poor prognosis
- genome wide
- mesenchymal stem cells
- low dose
- single cell
- cell therapy
- stem cells
- copy number
- binding protein
- gene expression
- acute lymphoblastic leukemia
- transcription factor
- newly diagnosed
- drug induced
- genome wide analysis