血小板減少を伴う自己免疫性疾患 : 自己免疫性血小板減少症,抗リン脂質抗体症候群(<特集>血小板減少症とアフェレシス)

概要

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Thrombocytopenia (<100,000/μl) is often associated with autoimmune disease, especially in systemic lupus erythematosus (SLE). Autoimmune thrombocytopenic purpura (ATP) and antiphospholipid syndrome (APS) are the major cause of such thrombocytopenia. Recent new findings about pathological mechanisms of ATP and APS provide a new approach for treatment. In ATP, the effectiveness of immunosuppressant drugs, anti-CD20 antibodies and thrombopoietin receptor antagonist for intractable cases has been reported. Although anticoagulant is still crucial in the APS treatment, new drugs targeting factor Ha (thrombin) and CD20 show remarkable effect. Furthermore, potential new drugs which target NF-/κb, p38-MAPK and factor Xa are under development. Plasmapheresis is effective for eliminating pathogenic molecules from the serum of patients with autoimmune disease associated thrombocytopenia. More complementary effect is expected synchronized with these new drugs.
2012-02-29