Autoimmune thrombocytopenic purpura and common variable immunodeficiency: analysis of 21 cases and review of the literature.

摘要: To describe the main characteristics and outcome of autoimmune thrombocytopenic purpura (AITP) in patients with common variable immunodeficiency (CVID), we analyzed data from 21 reviewed additional cases literature. be included this study, had to have CVID a previous history AITP platelet count or = 150 x 10(9)/L, partial response by >>50 10(9)/L an increase at least twofold initial level. The median diagnosis was 20 (range, 2-50 10(9)/L). age 23 years 1-51 yr), whereas 27 10-74 yr). diagnosed before onset only 4 (19%), 3 whom were being treated intravenous immunoglobulin (i.v.Ig) replacement therapy. more than 6 months after 13 (62%), 2 conditions concomitantly cases. Eleven (52%) 1 manifestation other AITP, among which hemolytic anemia (7 cases) neutropenia (5 preeminent. Seventeen (80%) received treatment for AITP; corticosteroids alone 7 (54%) achieved response; 8 i.v.Ig 1-2 g/kg combination steroids, leading short-term rate 50%. Four underwent splenectomy (2 complete responses, failures); splenectomies performed associated anemia. With mean follow-up 5.6 surgical procedure, none splenectomized life-threatening infection. 12 years, 13/21 (62%) treatment-free remission responses), (23%) while on prednisone < mg/day without azathioprine, patient still <50 10(9)/L. Five died time analysis; deaths related hemorrhage. Severe infections including fatal bacterial opportunistic occurred during AITP. In conclusion, (Evans syndrome) and/or neutropenia, is frequent CVID, not prevented substitutive Since frequently precedes testing levels should every Steroids seem same efficacy as idiopathic but increased risk severe must taken into consideration.