Successful Use of Rituximab in Refractory Idiopathic Thrombocytopenic Purpura in a Patient With Common Variable Immunodeficiency

摘要

Idiopathic thrombocytopenic purpura (ITP) is a common autoimmune disease in patients with common variable immunodeficiency (CVID). We describe a 36-year-old woman with CVID. The clinical course of her disease was complicated by bronchiectasis, antiphospholipid antibody syndrome, and portal vein thrombosis. She developed recurrent attacks of ITP refractory to high doses of corticosteroid, intravenousimmunoglobulin (IVIG), and splenectomy. She received a total of 5 doses of rituximab (375 mg/m2) and achieved an immediate and persistent response. Therapy was well tolerated. Her platelet count remained above 370 000/μL for 8 months of follow-up, despite repeated infections. During this period the patient remained off corticosteroids and on continuous IVIG replacement therapy.Key words: Rituximab. Idiopathic thrombocytopenic purpura. Common variable immunodeficiency.