ADAMTS13号
血栓性血小板减少性紫癜
医学
美罗华
血浆置换术
贝里穆马布
免疫学
胃肠病学
自身抗体
内科学
联合疗法
系统性红斑狼疮
B细胞激活因子
血小板
B细胞
抗体
疾病
作者
Reina Tsuda,Toshiki Kido,Ichiro Okada,Aoi Kobiyama,Masatoshi Kawataka,Miho Yamazaki,Ryoko Asano,Hiroyuki Hounoki,Koichiro Shinoda,Kazuyuki Tobe
摘要
ABSTRACT Patients with systemic lupus erythematosus (SLE) occasionally develop thrombotic thrombocytopenic purpura (TTP), which can be fatal. Here, we report a case of TTP developing 3 years after SLE remitted with rituximab (RTX) therapy. A 50-year-old woman was treated with RTX for marked immune thrombocytopenic purpura and autoimmune haemolytic anaemia due to SLE relapse. After induction of remission, she was treated with prednisolone alone without maintenance therapy with RTX. Approximately 3 years later, she was readmitted with marked thrombocytopenia and severe renal dysfunction. On admission, she was diagnosed with TTP for the first time based on severe reduction in a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) activity and detection of ADAMTS13 inhibitors. CD19+ B cells in the patient’s serum increased to 34%, suggesting that B cells had reactivated once the effect of RTX had subsided. The patient was successfully treated with plasmapheresis, glucocorticoid pulse therapy, and RTX. There are no previous reports of newly diagnosed TTP with ADAMTS13 inhibitor production after having achieved remission of SLE with RTX. Therefore, our report also discusses the potential mechanisms of production of new autoantibodies after B-cell depletion therapy.
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