Successful treatment of refractory thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus with combination of plasma exchange and low-dose rituximab

• Published in: Lupus Vol. 29; no. 14; pp. 1961 - 1967
• Main Authors: Ma, Wanlu, Bai, Wei, Wu, Xueyan, Zhao, Jiuliang, Li, Mengtao, Zeng, Xiaofeng
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.12.2020; Sage Publications Ltd
• Subjects: Apheresis; Corticosteroids; Immunomodulators; Immunosuppressive agents; Immunotherapy; L-Lactate dehydrogenase; Lactic acid; Lupus; Lymphocytes B; Methylprednisolone; Monoclonal antibodies; Non-Hodgkin's lymphoma; Patients; Peripheral blood; Platelets; Proteinuria; Purpura; Rituximab; Systemic lupus erythematosus; Targeted cancer therapy; Thrombocytopenia; Thrombocytopenic purpura; Thrombotic thrombocytopenic purpura; Thrombotic thrombocytopenic purpura; systemic lupus erythematosus; low-dose rituximab; prognosis
• ISSN: 0961-2033; 1477-0962; 1477-0962
• DOI: 10.1177/0961203320953473

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.