Multicenter experience in hematopoietic stem cell transplantation for serious complications of common variable immunodeficiency.

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. 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.

A group-based brief intervention for surgery patients with high-risk substance use.

OBJECTIVE: This study examined the feasibility and efficacy of a psychosocial intervention to address high-risk substance use in patients scheduled for elective surgery. METHOD: A group-format intervention, based on motivational interviewing principles, was provided prior to elective surgery to 107 participants with at-risk substance use, identified using the Alcohol Use Disorders Identification Test - Condensed (AUDIT-C) and self-report of illicit drug use. Patient satisfaction was assessed with an anonymous survey. Within-subject comparisons of substance use at baseline and at a postoperative follow-up evaluation were conducted. A control group of 67 surgery patients reporting high-risk substance use completed baseline assessments and received usual care. Medical outcomes and measures of utilization were compared between groups. RESULTS: Patient satisfaction with the brief intervention was high. A paired t test comparing average pre- and post-AUDIT-C scores showed significant reduction in substance use postsurgery (t = 9.94, P = .000), and participants reported intention to maintain reduced substance use levels. Between-group analyses revealed no significant differences in medical complications or utilization. CONCLUSION: Findings suggest that a group-based intervention for substance use disorder can be implemented as part of preoperative care and may contribute to decreased substance use prior to and following surgery. Further work is needed to identify methods to reduce adverse medical outcomes in surgical patients.