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An Pediatr (Barc). 2015 Feb;82(2):62-7. doi: 10.1016/j.anpedi.2014.04.014. Epub 2014 May 22.

[Survival analysis of hematopoietic stem cell transplantation in children with primary immunodeficiency in Spain].

[Article in Spanish]

Author information

1
Servicio de Oncología, Hematología y Trasplante de Progenitores Hematopoyéticos Pediátrica, Hospital Vall d'Hebron, Barcelona, España. Electronic address: rhladun@vhebron.net.
2
Servicio de Oncología y Hematología Pediátrica, Hospital Sant Pau i Santa Creu, Barcelona, España.
3
Servicio de Oncología y Hematología Pediátrica, Hospital del Niño Jesús, Madrid, España.
4
Servicio de Oncología y Hematología Pediátrica, Hospital La Paz, Madrid, España.
5
Servicio de Oncología, Hematología y Trasplante de Progenitores Hematopoyéticos Pediátrica, Hospital Vall d'Hebron, Barcelona, España.
6
Servicio de Oncología y Hematología Pediátrica, Hospital Regional Universitario Carlos Haya, Málaga, España.

Abstract

INTRODUCTION:

Children with primary immunodeficiency have severe life-threatening infections and a higher prevalence of autoimmune problems, allergy and lymphoproliferative disorders. Allogenic hematopoietic stem cell transplantation has been the only potentially curative option.

PATIENTS AND METHODS:

Patients with primary immunodeficiency underwent allogenic stem cell transplantation in the period 1985-2011, and registered in the Spanish Working Party for Bone Marrow Transplantation in Children.

RESULTS:

One hundred and fifty nine patients underwent 173 allogenic stem cell transplantations, of whom 97 had severe combined immunodeficiency, 30 with immune dysregulation disorders, 25 Wiskott-Aldrich syndrome, and 21 phagocyte disorders. The median patient age at diagnosis was 6 months (range: 17 days - 168 months) and the median patient age at transplant was 12 months (range: 1 month - 189 months). The donors were 30 (19%) identical siblings, 40 (25%) alternative family donors, and 89 (56%) unrelated donors. The source of stem cells was bone marrow in 68 (43%), cord blood in 52 (33%), and peripheral blood in 39 (24%). Ninety eight (61.6%) are alive, 57 (35.9%) died. Event-free survival at 10 years was 63%, with 90% for children transplanted from identical siblings, 36% for those transplanted from alternative family donors, and 66% for those transplanted from unrelated donors.

CONCLUSIONS:

The best results have been obtained with identical siblings, but other options may be considered.

KEYWORDS:

Hematopoietic stem cell transplantation; Immunosuppression; Inmunodeficiencias primarias; Inmunosupresión; Primary immunodeficiencies; Trasplante alogénico de progenitores hematopoyéticos

PMID:
24857430
DOI:
10.1016/j.anpedi.2014.04.014
[Indexed for MEDLINE]
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