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硼替佐米成功治疗镰状细胞病患者的严重难治性自身免疫性溶血性贫血(AIHA)

Successful Management of Severe and Refractory Autoimmune Hemolytic Anemia (AIHA) in a Sickle Cell Disease Patient With Bortezomib.

作者信息

Alanssari Mohammed A, Al-Sulaitti Elaf, Al-Sulaitti Zainab, Khalaf Asaad A, Dawood Qutaiba M

机构信息

Hematology, Basrah Health Directorate, Basrah, IRQ.

Hematology, Basrah Teaching Hospital, Basrah, IRQ.

出版信息

Cureus. 2024 Nov 30;16(11):e74840. doi: 10.7759/cureus.74840. eCollection 2024 Nov.

Abstract

Autoimmune hemolytic anemia (AIHA) is a multifactorial disease that causes immune-mediated red blood cell destruction, resulting in anemia and hemolysis symptoms. Despite a significant understanding of its pathogenesis, the precise causes of AIHA remain largely unclear and are thought to be multifactorial. In this paper, we presented a case of sickle cell anemia who developed severe AIHA that failed to maintain response to multiple treatment lines, including steroids, intravenous immunoglobulin, rituximab, and immune suppressive medications. However, a favorable response was achieved through the utilization of bortezomib. This report contributes to the expanding body of evidence regarding the efficacy of proteasome inhibitors in the management of severe and refractory autoimmune hemolysis in patients with sickle cell anemia.

摘要

自身免疫性溶血性贫血(AIHA)是一种多因素疾病,可导致免疫介导的红细胞破坏,从而引发贫血和溶血症状。尽管对其发病机制有了相当深入的了解,但AIHA的确切病因在很大程度上仍不清楚,被认为是多因素的。在本文中,我们报告了一例镰状细胞贫血患者,该患者发生了严重的AIHA,对包括类固醇、静脉注射免疫球蛋白、利妥昔单抗和免疫抑制药物在内的多种治疗方案均无反应。然而,通过使用硼替佐米取得了良好的疗效。本报告为蛋白酶体抑制剂在镰状细胞贫血患者严重难治性自身免疫性溶血治疗中的疗效提供了更多证据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be74/11684491/c27b91ea3ba1/cureus-0016-00000074840-i01.jpg

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