Successful treatment of refractory thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus with combination of plasma exchange and low-dose rituximab
| العنوان: | 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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| المؤلفون: | Wanlu Ma, Wei Bai, Xiaofeng Zeng, Mengtao Li, Xueyan Wu, Jiuliang Zhao |
| المصدر: | Lupus. 29:1961-1967 |
| بيانات النشر: | SAGE Publications, 2020. |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | Adult, medicine.medical_specialty, Adolescent, Thrombotic thrombocytopenic purpura, Disease, 030204 cardiovascular system & hematology, Gastroenterology, Drug Administration Schedule, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Refractory, hemic and lymphatic diseases, Internal medicine, Humans, Lupus Erythematosus, Systemic, Medicine, THROMBOCYTOPENIA PURPURA, Plasma Exchange, Purpura, Thrombotic Thrombocytopenic, business.industry, Systemic lupus, Low dose, medicine.disease, Antirheumatic Agents, 030220 oncology & carcinogenesis, Female, Rituximab, business, medicine.drug |
| الوصف: | 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/m2 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 × 109/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. |
| تدمد: | 1477-0962 0961-2033 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5a29c279bb6373321cfc49e5352dcb51Test https://doi.org/10.1177/0961203320953473Test |
| حقوق: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....5a29c279bb6373321cfc49e5352dcb51 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14770962 09612033 |
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