Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-Aldrich

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 i...

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Autores principales:	Wietstruck P,María Angélica, Zúñiga C,Pamela, Talesnik G,Eduardo, Méndez R,Cecilia, Barriga C,Francisco
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2007
Materias:	Hematopoietic stem cells Transplantation, homologous
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007000700013
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spelling	oai:scielo:S0034-988720070007000132007-09-06Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-AldrichWietstruck P,María AngélicaZúñiga C,PamelaTalesnik G,EduardoMéndez R,CeciliaBarriga C,Francisco Hematopoietic stem cells Transplantation, homologous 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 occurinfo:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.135 n.7 20072007-07-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007000700013es10.4067/S0034-98872007000700013
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Hematopoietic stem cells Transplantation, homologous
spellingShingle	Hematopoietic stem cells Transplantation, homologous Wietstruck P,María Angélica Zúñiga C,Pamela Talesnik G,Eduardo Méndez R,Cecilia Barriga C,Francisco Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-Aldrich
description	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
author	Wietstruck P,María Angélica Zúñiga C,Pamela Talesnik G,Eduardo Méndez R,Cecilia Barriga C,Francisco
author_facet	Wietstruck P,María Angélica Zúñiga C,Pamela Talesnik G,Eduardo Méndez R,Cecilia Barriga C,Francisco
author_sort	Wietstruck P,María Angélica
title	Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-Aldrich
title_short	Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-Aldrich
title_full	Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-Aldrich
title_fullStr	Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-Aldrich
title_full_unstemmed	Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-Aldrich
title_sort	trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de wiskott-aldrich
publisher	Sociedad Médica de Santiago
publishDate	2007
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007000700013
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Trasplante alogénico de precursores hematopoyéticos en pacientes con síndrome de Wiskott-Aldrich

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