SciELO - Scientific Electronic Library Online

vol.135 número7Manifestaciones neurológicas de la granulomatosis de Wegener: Caso clínicoQuiste broncogénico retroperitoneal: Caso clínico índice de autoresíndice de materiabúsqueda de artículos
Home Pagelista alfabética de revistas  

Servicios Personalizados




Links relacionados


Revista médica de Chile

versión impresa ISSN 0034-9887


WIETSTRUCK P, María Angélica et al. Hematopoietic stem cell transplantation for patients with Wiskott-Aldrich syndrome. Rev. méd. Chile [online]. 2007, vol.135, n.7, pp.917-923. ISSN 0034-9887.

Background: Wiskott-Aldrich syndrome (WAS) is an X linked congenital disease that presents as eczema, thrombocytopenia and immune deficiency. The only curative procedure for this illness is hematopoietic stem cell transplant (HSCT), preferably from a healthy HLA identical sibling donor. Cord blood is becoming an excellent alternative as stem cell source from unrelated donors. Aim: To report our experience with HSCT in children with WAS. Patients and methods: Six boys with WAS diagnosed at 1 to 6 months of age were transplanted at our institution. All of them developed eczema and thrombocytopenia. Two had episodes of severe bleeding and three had repetitive infections (two with recurrent pulmonary infections and one a recurrent otitis). Three patients had a positive family history. Two received HSCT from sibling donors and four from unrelated cord blood donors at 7 months to 4 years of age. Results: AH 6 patients had full hematopoietic engraftment after transplantation. Three had mild chronic graft-versus- host disease which responded to immune suppressive therapy. One patient died of cytomegalovirus related pneumonia 111 days after grafting. The other 5 patients are alive and healthy 11 to 104 months after transplantation. Conclusions: HSCT is an effective treatment for patients with WAS. The procedure should be done as soon as diagnosis is confirmed and before life threatening infections occur

Palabras clave : Hematopoietic stem cells; Transplantation, homologous.

        · texto en Español     · Español ( pdf )


Creative Commons License Todo el contenido de esta revista, excepto dónde está identificado, está bajo una Licencia Creative Commons