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Post-Immunoglobuin Incidence of ITP and AIHA in patients with CVID
Sunday, March 5, 2017: 2:00 PM
Rooms B308-B309 (Georgia World Congress Center, Building B)
Kara Beth McNamara, MD,
Rationale: In patients with common variable immunodeficiency (CVID), autoimmune hematologic disorders such as immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA), are commonly seen, with an incidence of 7-11%. As immunoglobulin replacement is used as a treatment modality for CVID as well as acute ITP (and occasionally AIHA), it has been hypothesized that immunoglobulin therapy may have a protective effect against the development of such cytopenias in the CVID population.  

Methods: A retrospective chart review was performed. Charts were obtained of CVID patients receiving immunoglobulin replacement therapy in the past 20 years at a tertiary care referral center. The subset with an additional diagnosis or either ITP, AIHA, or both were identified and reviewed for the dates of immunoglobulin therapy initiation and dates of ITP/AIHA diagnosis.

Results: A total of 795 CVID patient were identified. Of these, there were 58 cases of newly diagnosed ITP, AIHA, or both. Of these 58 cases, 29 occurred prior to initiation of immunoglobulin replacement, 28 occurred post initiation, and 1 was not included in analysis as the  timeline could not be delineated.  The overall incidence of ITP/AIHA  pre- immunoglobulin replacement therapy was 3.6 % and the incidence post-immunoglobulin therapy was 3.5%.

Conclusions: The overall incidence of ITP/AIHA was similar in CVID patients pre- and post- immunoglobulin replacement. Although our sample size is not large, our review suggests that immunoglobulin replacement therapy may not be protective against autoimmune cytopenias in the CVID population. Future studies involving a larger cohort or immunomodulating doses may be warranted.