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首页> 外文期刊>Lupus >Successful treatment of refractory thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus with combination of plasma exchange and low-dose rituximab
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Successful treatment of refractory thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus with combination of plasma exchange and low-dose rituximab

机译：成功治疗耐火性血栓形成血小板紫癜，其血浆交换组合和低剂量蓖麻血红蛋白

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摘要

Objectives Thrombotic thrombocytopenia purpura (TTP) associated with systemic lupus erythematous (SLE) (i.e., SLE-TTP) is a rare life-threatening disease often requiring intensive immunosuppressive agents, in addition to high-dose corticosteroids and plasma exchange (PEX). The optimal therapy of rituximab is unclear, but 375 mg/m(2)weekly for 4 weeks is the usual practice, adopted from regimens for non-Hodgkin's lymphoma. We reported two cases of refractory SLE-TTP that showed good efficacy and prognosis with combination of methylprednisolone (MP) pulse, plasma exchange and low-dose rituximab (100 mg weekly for 4 weeks) treatment. Methods Clinical data and treatment outcomes were reviewed of two patients diagnosed with refractory SLE-TTP at Peking Union Medical College Hospital between July 2017 and July 2018. Results Both patients had SLE and presented with microangiopathic anemia and thrombocytopenia. Laboratory assays revealed high anti-nuclear antibody titers, reduced complement 3 and 4 levels, proteinuria, significantly elevated lactate dehydrogenase, schistocytes on peripheral blood smear, low ADAMTS13 activity, and the presence of ADAMTS13 inhibitor. In both patients, platelet counts remained below 50 x 10(9)/L after MP pulse and 6 PEXs, confirming the diagnosis of refractory SLE-TTP. Low-dose rituximab (100 mg weekly for 4 weeks) was administered in both cases, resulting in normalization of platelet counts and significant reductions in B-lymphocyte counts. No TTP relapse or SLE flare occurred during 24 months of follow-up. Conclusions Our cases confirmed the efficacy and good follow-up outcomes of low-dose rituximab treatment (100 mg weekly for 4 weeks) for refractory SLE-TTP.
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  • 来源
    《Lupus》 |2020年第14期|共7页
  • 作者

    Ma Wanlu; Bai Wei; Wu Xueyan; Zhao Jiuliang; Li Mengtao; Zeng Xiaofeng;

  • 作者单位

    Chinese Acad Med Sci Peking Union Med Coll Hosp PUMCH Dept Endocrinol Beijing Peoples R China;

    Chinese Acad Med Sci Peking Union Med Coll Hosp PUMCH Dept Rheumatol Beijing Peoples R China;

    Chinese Acad Med Sci Peking Union Med Coll Hosp PUMCH Dept Endocrinol Beijing Peoples R China;

    Chinese Acad Med Sci Peking Union Med Coll Hosp PUMCH Dept Rheumatol Beijing Peoples R China;

    Chinese Acad Med Sci Peking Union Med Coll Hosp PUMCH Dept Rheumatol Beijing Peoples R China;

    Chinese Acad Med Sci Peking Union Med Coll Hosp PUMCH Dept Rheumatol Beijing Peoples R China;

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  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类 免疫性疾病;
  • 关键词

    Thrombotic thrombocytopenic purpura; systemic lupus erythematosus; low-dose rituximab; prognosis;

    机译：血栓形成血小板减少紫癜;Systemic Lupus红斑狼疮;低剂量蓖麻毒素;预后;

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