A case of common variable immunodeficiency disease associated with idiopathic thrombocytopenic purpura

A rare case of common variable immunodeficiency disease who developed idiopathic thrombocytopenic purpura was reported. In October 1981, a 29-year-old woman was admitted to the Keio University Hospital because of aseptic meningitis. On this occasion, she was found to have hypogammaglobulinemia (IgG 277 mg/dl/, IgG 22 mg/dl/, IgM 18 mg/dl/). The in vitro culture experiments indicated that defective B cell function and activated suppressor T cells might account for hypogammaglobulinemia observed in this patient. A diagnosis of common variable immunodeficiency disease was made and monthly supplementation therapy by gammaglobulin was started. On July 1985, she was readmitted because of severe thrombocytopenia (10,000/mm³). Physical examination revealed multiple purpura on her upper and lower extremities. Bone marrow examination showed normocellularity with normal number of megakaryocytes (70/mm³). Tests for circulating immune complexes were negative and platelet bound IgG (PBIgG) was within normal limits. Titers of antibodies to viral antigens (EB, cytomegalo, rubella, coxackie, measles, influenza) were not elevated. Treatment by prednisolone (60 mg/day) was ineffective and high dose intravenous immunoglobulin resulted in only transient increase of platelet counts. From the 17th hospital day, the vincristine slow infusion therapy was started and remarkable recovery of thrombocytopenia was obtained. Although it might be difficult to determine whether ITP in this case was of the acute or chronic type, we could not find an established case report of common variable immunodeficiency disease complicated with ITP. The mechanisms of action of slowly infused vincristine in ITP and pathogenetic mechanisms of ITP occurred in an immunodeficient patient were discussed.