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Multicenter experience in hematopoietic stem cell transplantation for serious complications of common variable immunodeficiency.
- Source :
-
The Journal of allergy and clinical immunology [J Allergy Clin Immunol] 2015 Apr; Vol. 135 (4), pp. 988-997.e6. Date of Electronic Publication: 2015 Jan 14. - Publication Year :
- 2015
-
Abstract
- Background: Common variable immunodeficiency (CVID) is usually well controlled with immunoglobulin substitution and immunomodulatory drugs. A subgroup of patients has a complicated disease course with high mortality. For these patients, investigation of more invasive, potentially curative treatments, such as allogeneic hematopoietic stem cell transplantation (HSCT), is warranted.<br />Objective: We sought to define the outcomes of HSCT for patients with CVID.<br />Methods: Retrospective data were collected from 14 centers worldwide on patients with CVID receiving HSCT between 1993 and 2012.<br />Results: Twenty-five patients with CVID, which was defined according to international criteria, aged 8 to 50 years at the time of transplantation were included in the study. The indication for HSCT was immunologic dysregulation in the majority of patients. The overall survival rate was 48%, and the survival rate for patients undergoing transplantation for lymphoma was 83%. The major causes of death were treatment-refractory graft-versus-host disease accompanied by poor immune reconstitution and infectious complications. Immunoglobulin substitution was stopped in 50% of surviving patients. In 92% of surviving patients, the condition constituting the indication for HSCT resolved.<br />Conclusion: This multicenter study demonstrated that HSCT in patients with CVID was beneficial in most surviving patients; however, there was a high mortality associated with the procedure. Therefore this therapeutic approach should only be considered in carefully selected patients in whom there has been extensive characterization of the immunologic and/or genetic defect underlying the CVID diagnosis. Criteria for patient selection, refinement of the transplantation protocol, and timing are needed for an improved outcome.<br /> (Copyright © 2014 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)
- Subjects :
- Adolescent
Adult
Cause of Death
Child
Common Variable Immunodeficiency complications
Common Variable Immunodeficiency mortality
Female
Follow-Up Studies
Graft Survival
Graft vs Host Disease etiology
Graft vs Host Disease prevention & control
Humans
Male
Middle Aged
Retrospective Studies
Transplantation Conditioning
Treatment Outcome
Young Adult
Common Variable Immunodeficiency therapy
Hematopoietic Stem Cell Transplantation adverse effects
Hematopoietic Stem Cells
Subjects
Details
- Language :
- English
- ISSN :
- 1097-6825
- Volume :
- 135
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- The Journal of allergy and clinical immunology
- Publication Type :
- Academic Journal
- Accession number :
- 25595268
- Full Text :
- https://doi.org/10.1016/j.jaci.2014.11.029