Multicenter experience in hematopoietic stem cell transplantation for serious complications of common variable immunodeficiency.
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Wehr Claudia, Gennery Andrew R, Lindemans Caroline, Schulz Ansgar, Hoenig Manfred, Marks Reinhard, Recher Mike, Gruhn Bernd, Holbro Andreas, Heijnen Ingmar, Meyer Deborah, Grigoleit Goetz, Einsele Hermann, Baumann Ulrich, Witte Thorsten, Sykora Karl-Walter, Goldacker Sigune, Regairaz Lorena, Aksoylar Serap, Ardeniz Ömur, Zecca Marco, Zdziarski Przemyslaw, Meyts Isabelle, Matthes-Martin Susanne, Imai Kohsuke, Kamae Chikako, Fielding Adele, Seneviratne Suranjith, Mahlaoui Nizar, Slatter Mary A, Güngör Tayfun, Arkwright Peter D, van Montfrans Joris, Sullivan Kathleen E, Grimbacher Bodo, Cant Andrew, Peter Hans-Hartmut, Finke Juergen, Gaspar H Bobby, Warnatz Klaus, Rizzi Marta, Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immunodeficiency
Аннотация
OBJECTIVE: We sought to define the outcomes of HSCT for patients with CVID.
METHODS: Retrospective data were collected from 14 centers worldwide on patients with CVID receiving HSCT between 1993 and 2012.
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.
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.