抗胸腺细胞球蛋白联合环孢素A治疗儿童重型再生障碍性贫血诱发毛细血管渗透综合征1例

doi:10.3969/j.issn.1004-583X.2023.09.008

摘要/Abstract

摘要：

目的提高临床医生对毛细血管渗透综合征的认识,减少漏诊、误诊,改善预后。方法回顾性分析我院收治的1例抗胸腺细胞球蛋白(anti-thymocyte globulin, ATG) 联合环孢素A(cyclosporine A, CSA)治疗重型再生障碍性贫血(severe aplastic anemia,SAA)诱发毛细血管渗漏综合征(capillary leak syndrome,CLS)患儿的临床资料,总结临床特征及诊疗经过,并结合相关文献进行分析。结果应用免疫抑制剂疗法治疗后第13天患儿发生CLS,临床特征包括进行性呼吸困难、低血压、周身水肿、低蛋白血症及多浆膜腔积液等。经激素冲击、羟乙基淀粉扩容、呋塞米利尿脱水及输血支持治疗后,症状及体征逐渐好转。结论 CLS是SAA患者应用ATG联合CSA免疫抑制剂疗法的罕见并发症,且有致死风险,需引起重视。早识别、早诊断、早治疗可改善患者预后。

关键词: 贫血, 再生障碍性, 抗淋巴细胞血清, 环孢菌素 A, 毛细血管渗漏综合征

Abstract:

Objective To improve clinicians' understanding of capillary infiltration syndrome (CAS), to reduce missed diagnosis and misdiagnosis, and improve the prognosis. Methods Clinical data of a child with severe aplastic anemia (SAA)-induced CAS treated with anti-thymocyte globulin (ATG) combined with cyclosporine A (CSA) were retrospectively analyzed. The clinical characteristics and treatment course were summarized. Relevant literatures were reviewed as well. Results In this study, we reported a SAA child with CLS on the 13 days of the immunosuppressant treatment. The clinical features included progressive dyspnea, hypotension, peripheral edema, hypoproteinemia, and multiple plasma cavity effusions. After hormonal shock, hydroxyethyl starch expansion, furosemide diuretic dehydration and blood transfusion support, the symptoms and signs were gradually improved. Conclusion CLS is a rare complication during the application of ATG combined with immunosuppressive therapy of CSA in SAA patients, which has the risk of death that should be well concerned. Early recognition, diagnosis and treatment can improve the prognosis.

Key words: anemia, aplastic, antilymphocyte serum, cyclosporine A, capillary leak syndrome

中图分类号:

R556.5

谷翠红, 王阳, 林丽, 王丽红, 张志华. 抗胸腺细胞球蛋白联合环孢素A治疗儿童重型再生障碍性贫血诱发毛细血管渗透综合征1例[J]. 临床荟萃, 2023, 38(9): 819-322.

Gu Cuihong, Wang Yang, Lin Li, Wang Lihong, Zhang Zhihua. Anti-thymocyte globulin combined with cyclosporine A in the treatment of severe aplastic anemia-induced caplillary leak syndrome in children: A case report[J]. Clinical Focus, 2023, 38(9): 819-322.

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链接本文: https://huicui.hebmu.edu.cn/CN/10.3969/j.issn.1004-583X.2023.09.008

https://huicui.hebmu.edu.cn/CN/Y2023/V38/I9/819

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