HSCT transplantation in patients with CVID

Posted by & filed under Part 20: IMMUNE AND DEFENSE SYSTEMS.

Wehr et al explored the outcomes of hematopoietic stem cell transplantation (HSCT) in patients with Common variable immunodeficiency (CVID). Based on retrospective data collected from 14 centers worldwide, 25 patients with CVID, who received HSCT between 1993 and 2012, were identified. These patients’ ages ranged from 8 to 50 years at the time of transplantation. The overall survival rate was 48%, and the survival rate for patients undergoing transplantation for lymphoma was 83%. Graft-versus-host disease associated with infectious complications was the principal cause of death. In 92% of surviving patients, the condition constituting the indication for HSCT resolved.

In conclusion, HSCT in patients with CVID was successful in most surviving patients. However, due to the high mortality associated with the procedure, only patients who do not respond to immunoglobulin substitution and immunomodulatory drugs should be considered for HSCT.

Multicenter experience in hematopoietic stem cell transplantation for serious complications of common variable immunodeficiency. Wehr et al; the Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immunodeficiency. J Allergy Clin Immunol. 2015 Jan 14. pii: S0091-6749(14)01732-1.  PMID: 25595268

posted by Yannis Trakadis, MD

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