Department of Rheumatology, Xiangya Hospital, Central South University, Changsha, China.
Department of Blood Transfusion, Xiangya Hospital, Central South University, Changsha, China.
Front Immunol. 2022 Jun 10;13:861719. doi: 10.3389/fimmu.2022.861719. eCollection 2022.
Severe autoimmune haemolytic anaemia (AIHA) in systemic lupus erythematosus (SLE) patients could be life-threatening and formidable, especially in those nonresponsive to glucocorticoids (GCs) and immunosuppressants (ISAs). Whole-blood exchange transfusion (WBE), with plasma exchange and pathogenic cell removal as well as healthy red blood cell transfusion, could be beneficial. The objective of this study was to investigate the efficacy and safety of WBE in combination with GCs/ISAs.
In this retrospective study, the clinical data of 22 refractory severe SLE-AIHA inpatients between February 2016 and February 2021 were collected and analysed, among whom 14 patients had received WBE and were compared with those treated with typical second-line therapy of intravenous immunoglobulin and/or rituximab (IVIG/RTX).
Among the 22 severe refractory SLE-AIHA patients, eight patients received IVIG and/or RTX without WBE (group 1, IVIG/RTX, = 8), seven patients were given WBE without IVIG/RTX (group 2, WBE alone, = 7), and seven patients who failed initial IVIG/RTX therapy were given sequential WBE therapy (group 3 IVIG/RTX→WBE, = 7). Fourteen patients had accepted WBE treatment regardless of prior IVIG/RTX usage (group 2 + 3, WBE ± IVIG/RTX, = 14). On days 1, 3, 5, and 7 after corresponding therapies, patients of groups 2, 3, and 2 + 3 showed significantly higher levels of haemoglobin (Hb) than patients of group 1. Compared with patients of group 1, patients of groups 2, 3, and 2 + 3 took less time to reach and maintain Hb ≥60 g/L from baseline. Groups 2 and 2 + 3 consumed a lower dose of GCs than group 1 to reach and maintain Hb ≥60 g/L from baseline. Group 1 experienced longer hospital stays than group 2, and group 3's cost of hospitalisation is more than groups 1 and 2. Hb <40 g/L may be a key indicative factor for initiating WBE remedy therapy as IVIG/RTX may not be effective enough in 48-72 h in those patients with refractory severe SLE-AIHA. No severe adverse effects were observed in the WBE group.
WBE could be a safe and beneficial alternative therapy for refractory severe SLE-AIHA.
系统性红斑狼疮（SLE）患者发生严重自身免疫性溶血性贫血（AIHA）可能危及生命，尤其是那些对糖皮质激素（GCs）和免疫抑制剂（ISAs）无反应的患者。全血置换输血（WBE）可联合血浆置换和致病性细胞清除以及输注健康的红细胞，这可能有益。本研究旨在探讨 WBE 联合 GCs/ISAs 的疗效和安全性。
本回顾性研究收集了 2016 年 2 月至 2021 年 2 月期间 22 例难治性严重 SLE-AIHA 住院患者的临床资料，并进行了分析，其中 14 例患者接受了 WBE 治疗，并与接受典型二线治疗静脉免疫球蛋白和/或利妥昔单抗（IVIG/RTX）的患者进行了比较。
在 22 例严重难治性 SLE-AIHA 患者中，8 例患者未接受 WBE 而接受了 IVIG/RTX（组 1，IVIG/RTX， = 8），7 例患者仅接受 WBE 治疗（组 2，单独 WBE， = 7），7 例初始 IVIG/RTX 治疗失败的患者接受了序贯 WBE 治疗（组 3，IVIG/RTX→WBE， = 7）。无论是否使用 IVIG/RTX，14 例患者均接受了 WBE 治疗（组 2 + 3，WBE ± IVIG/RTX， = 14）。在相应治疗后的第 1、3、5 和 7 天，组 2、3 和 2 + 3 的患者血红蛋白（Hb）水平明显高于组 1。与组 1 相比，组 2、3 和 2 + 3 的患者达到并维持基线时 Hb≥60g/L 所需的时间更短。与组 1 相比，组 2 和 2 + 3 达到并维持 Hb≥60g/L 所需的 GCs 剂量更低。组 1 的住院时间长于组 2，组 3 的住院费用高于组 1 和组 2。Hb<40g/L 可能是启动 WBE 治疗的关键指标，因为对于难治性严重 SLE-AIHA 患者，IVIG/RTX 在 48-72 小时内可能效果不佳。WBE 组未观察到严重不良反应。
WBE 可能是治疗难治性严重 SLE-AIHA 的一种安全有效的替代疗法。
