医学
美罗华
血栓性血小板减少性紫癜
内科学
微血管病性溶血性贫血
胃肠病学
分裂细胞
耐火材料(行星科学)
ADAMTS13号
来氟米特
免疫学
血小板
类风湿性关节炎
淋巴瘤
天体生物学
物理
作者
Wanlu Ma,Wei Bai,Xueyan Wu,Jiuliang Zhao,Mengtao Li,Xiaofeng Zeng
出处
期刊:Lupus
[SAGE]
日期:2020-08-24
卷期号:29 (14): 1961-1967
被引量:7
标识
DOI:10.1177/0961203320953473
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 × 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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