严重自身免疫性溶血性贫血合并遗传性球形红细胞增多症经糖皮质激素和环孢素成功治疗：病例报告。
Severe autoimmune hemolytic anemia complicating hereditary spherocytosis treated successfully with glucocorticoids and cyclosporine: a case report.
机构信息
Department of Blood Transfusion, China-Japan Friendship Hospital, Beijing, People's Republic of China.
Clinical Laboratory, China-Japan Friendship Hospital, Beijing, People's Republic of China.
出版信息
Hematology. 2023 Dec;28(1):2235832. doi: 10.1080/16078454.2023.2235832.
BACKGROUND
Severe autoimmune hemolytic anemia complicating hereditary spherocytosis is life threatening and has not been described in a case report. Here, we report a case in which this intractable disease was treated successfully with glucocorticoids and cyclosporine.
CASE PRESENTATION
A 25-year-old female patient with hereditary spherocytosis developed severe autoimmune hemolytic anemia after respiratory syncytial virus infection. Her hemoglobin level was 26 g/L and various anti-red blood cell antibodies were detected in her serum, making blood matching difficult. Glucocorticoid monotherapy was ineffective. With the addition of cyclosporine (50 mg/12 h), the patient's hemoglobin level increased significantly and the symptoms associated with anemia were greatly relieved.
CONCLUSION
In patients with severe autoimmune hemolytic anemia, especially when the presence of multiple anti-red blood cell antibodies and alloantibodies interferes with blood matching, a glucocorticoid-cyclosporine regimen may be tried.
背景
遗传性球形红细胞增多症并发严重自身免疫性溶血性贫血具有生命威胁，尚未有病例报告对此进行描述。在此，我们报告了一例使用糖皮质激素和环孢素成功治疗这种难治性疾病的病例。
病例介绍
一名 25 岁女性遗传性球形红细胞增多症患者在呼吸道合胞病毒感染后发生严重自身免疫性溶血性贫血。其血红蛋白水平为 26 g/L，血清中检测到多种抗红细胞抗体，导致配血困难。糖皮质激素单药治疗无效。加用环孢素（50mg/12h）后，患者的血红蛋白水平显著升高，贫血相关症状得到极大缓解。
结论
对于严重自身免疫性溶血性贫血患者，尤其是当存在多种抗红细胞抗体和同种抗体干扰配血时，可尝试糖皮质激素-环孢素方案。
Zotero 插件