Characteristics and Outcome of Autoimmune Thrombocytopenic Purpura (AITP) in Patients with Common Variable Immunodeficiency (CVI): Analysis of 21 Cases. Session Type: Poster Session 180-I

Marc Michel, Valerie Chanet, Olivier Hermine, Lionel Galicier, Philippe Bierling, Bertrand Godeau

Author address: 

Medecine Interne, Hopital Henri Mondor, Creteil, France; Hematologie, Hopital Necker, Paris, France; Immuno-Hematologie, Hopital Saint-Louis, Paris, France; Etablissement Francais du Sang, Creteil, France

Abstract: 

Common variable immunodeficiency (CVI) is a heterogeneous syndrome characterized by persistent hypogammaglobulinemia and recurrent bacterial infections. Besides recurrent infections, approximately 20 to 25% of patients (pts) with CVI develop autoimmune disease among which autoimmune cytopenias are preeminent. To describe the characteristics and outcome of AITP in pts with CVI, the data from 21 pts (12 men and 9 women) was analyzed. To be included the pts had to have: 1) a CVI, defined by an immunoglobulin (Ig) level 50 x 10 9/L. Acute AITP was defined by a treatment-free CR within 6 months of AITP onset. The estimated prevalence of AITP was 20% (21 of the 105 patients diagnosed with CVI during the study period). At AITP diagnosis, the median plt count was 20 x 10 9/L [2-50], and the median age was 23 years [1-51]. CVI was diagnosed before the onset of AITP in only 4 cases (19%), 3 of whom being already treated with intravenous immunoglobulins (IVIG) replacement therapy. CVI was diagnosed more than 6 months after AITP in 13 cases (62%) whereas the 2 conditions were diagnosed concomitantly in 4 cases. Eleven pts (52%) had at least one autoimmune manifestation other than AITP, among which autoimmune hemolytic anemia (AIHA) (7 cases) and autoimmune neutropenia (5 cases) were preeminent. 17/21 pts (81%) received at least one treatment for AITP. The response-rates (PR or CR) to corticosteroids and IVIG (1-2 g/kg) were 54% and 50% respectively. Four pts underwent splenectomy (2 CR and 2 failures), 2 additional splenectomies were performed for an associated AIHA. After a mean follow-up of 5.6 years following the procedure, none of the 6 splenectomized pts experienced a life-threatening infection. Although the course of ITP was chronic (> 6 months) in all pts but 2 (90%); after a median follow-up of 12 years after AITP onset, 62% of them were in treatment-free remission, 23% were in remission while on prednisone 20 mg/day azathioprine and only 1 pt had a plt count
2003

abstract No: 

1068

Full conference title: 

American Society of Hematology 45th Annual Meeting
    • ASH 45th (2003)