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探索蛋白A免疫吸附疗法治疗伴有高IgG4血症的自身免疫性溶血性贫血

Exploring Protein A Immunoadsorption for Autoimmune Hemolytic Anemia with Hyper-IgG4emia.

作者信息

Wang Jianwen, Li Yuxin, Wang Xinran, Yang Xinyu, Yang Yeyi, Zhang Hao

机构信息

Department of Nephropathy, Third Xiangya Hospital of Central South University, Changsha, Hunan, China.

The Critical Kidney Disease Research Center of Central South University, Changsha, Hunan, China.

出版信息

Am J Case Rep. 2025 Apr 11;26:e945746. doi: 10.12659/AJCR.945746.

Abstract

BACKGROUND Autoimmune hemolytic anemia (AIHA) is a hemolytic anemia characterized by autoantibodies against red blood cells. Patients with AIHA can have 4 subtypes of IgG-type red blood cell antibodies: IgG1, IgG2, IgG3, and IgG4. The development of this disease is closely related to IgG 1 and IgG 3, and the combination with high IgG4 is rare. A patient with autoimmune hemolytic anemia who had a poor response to the steroid combined with immunosuppressive regimen (methylprednisolone and cyclophosphamide) received 4 sessions of protein A immunosorbent therapy with good results and is still under continued follow-up. CASE REPORT A 60-year-old woman had recurrent dizziness, weakness, darkening of urine, and jaundice for 2 months. Five years ago, she underwent a lymph node biopsy for "pelvic lymph node enlargement", which indicated "reactive lymph node hyperplasia". Bone marrow aspiration indicated "myelodysplasia", excluding leukemia and plasma cell disease. This patient was first treated with the steroid combination immunosuppressive regimen (methylprednisolone and cyclophosphamide), but she had a poor outcome and an increase in progressive anemia. She was treated with methylprednisolone and cyclophosphamide combined with protein A immunoadsorption therapy. She responded well and her clinical symptoms improved after 2 weeks of treatment. Her malaise was significantly reduced, jaundice decreased, Hb rose to 76 g/L, and IgG4 decreased to 12.4 g/L. At the outpatient review after 2 months, the patient's clinical symptoms had disappeared, hemoglobin (Hb) increased to 136 g/L, and IgG4 decreased to 6.72 g/L. CONCLUSIONS Protein A immunosorbent therapy may be an effective treatment option for patients with AIHA who have a poor response to conventional therapy.

摘要

背景

自身免疫性溶血性贫血(AIHA)是一种以针对红细胞的自身抗体为特征的溶血性贫血。AIHA患者可出现4种IgG型红细胞抗体亚型:IgG1、IgG2、IgG3和IgG4。该疾病的发生与IgG1和IgG3密切相关,与高IgG4合并的情况罕见。一名自身免疫性溶血性贫血患者对类固醇联合免疫抑制方案(甲泼尼龙和环磷酰胺)反应不佳,接受了4次蛋白A免疫吸附治疗,效果良好,目前仍在持续随访中。病例报告:一名60岁女性反复出现头晕、乏力、尿色加深和黄疸2个月。5年前,她因“盆腔淋巴结肿大”接受了淋巴结活检,结果显示“反应性淋巴结增生”。骨髓穿刺显示“骨髓发育异常”,排除白血病和浆细胞病。该患者最初接受类固醇联合免疫抑制方案(甲泼尼龙和环磷酰胺)治疗,但效果不佳,进行性贫血加重。她接受了甲泼尼龙、环磷酰胺联合蛋白A免疫吸附治疗。治疗2周后反应良好,临床症状改善。她的不适明显减轻,黄疸减退,血红蛋白升至76 g/L,IgG4降至12.4 g/L。2个月后的门诊复查显示,患者临床症状消失,血红蛋白(Hb)升至136 g/L,IgG4降至6.72 g/L。结论:对于对传统治疗反应不佳的AIHA患者,蛋白A免疫吸附治疗可能是一种有效的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fe3a/11997903/f8bc076e68ca/amjcaserep-26-e945746-g001.jpg

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