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使用isatuximab、泊马度胺和地塞米松有效治疗难治性单克隆丙种球蛋白病相关纯红细胞再生障碍性贫血。

Effective treatment of refractory monoclonal gammopathy-associated pure red cell aplasia with isatuximab, pomalidomide and dexamethasone.

作者信息

Michel Christian Sebastian, Fehr Eva Marie, Nolte Hildegard, Beck Joachim, Kreft Andreas, Rauschkolb-Olk Katharina Theresa, Jacobi Bjoern, Theobald Matthias, Munder Markus

机构信息

Department of Hematology and Medical Oncology University Medical Center of the Johannes Gutenberg University Mainz Germany.

Department of Pathology University Medical Center of the Johannes Gutenberg University Mainz Germany.

出版信息

EJHaem. 2024 Jun 17;5(4):838-841. doi: 10.1002/jha2.964. eCollection 2024 Aug.

Abstract

Monoclonal gammopathy-associated pure red cell aplasia (MG-PRCA) is characterized by the absence or pronounced hypoplasia of erythroid precursors in the bone marrow, causing reticulocytopenia and a normocytic, normochromic anaemia in a patient with a monoclonal plasma cell dyscrasia. We report here on the successful treatment of MG-PRCA with isatuximab, pomalidomide, and dexamethasone after multiple lines of immunosuppressive and anti-plasma cell-directed treatments.

摘要

单克隆丙种球蛋白病相关纯红细胞再生障碍性贫血(MG-PRCA)的特征是骨髓中红系前体细胞缺失或显著发育不全,导致单克隆浆细胞异常增生患者出现网织红细胞减少和正细胞正色素性贫血。我们在此报告,在经过多线免疫抑制和抗浆细胞定向治疗后,使用isatuximab、泊马度胺和地塞米松成功治疗MG-PRCA的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5226/11327726/b691dadc1aed/JHA2-5-838-g002.jpg

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