首页|超溶血综合征患者的诊断分析和临床治疗策略——附1例报道

超溶血综合征患者的诊断分析和临床治疗策略——附1例报道

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目的 分析 1 例超溶血综合征(hyperhemolysis syndrome,HHS)反应的病例,探讨该疾病的实验室检查方案和临床治疗策略。方法 对患者做血型鉴定、直抗、抗筛、抗体鉴定等血清学试验,监测患者血常规,以及乳酸脱氢酶(lactate dehydrogenase,LDH)、胆红素等溶血指标,监测患者溶血的变化和发展情况。通过基因分型的方法对临床常见的 32 个稀有血型抗原进行检测,辅助分析抗体鉴定结果的准确性。结果 患者血清中存在抗-E并合并有自身抗体,输血后LDH、胆红素明显升高,血红蛋白(Hb)急剧下降,远远低于输血前的,出现血红蛋白尿等独特表现,经过大剂量静注免疫球蛋白和皮质类固醇类激素治疗,完全康复。结论 HHS是比较少见潜在致命性疾病,主要表现为溶血,严重贫血,且输血后血红蛋白水平往往低于输血前。本名患者具有输血后超溶血综合反应的典型临床表现,经过及时正确的治疗患者完全康复出院,为该类疾病的诊断和治疗积累了很好的临床经验。
Diagnostic analysis and clinical treatment strategies for patients with hyperhemolytic syndrome:a case report
Objective To analyze a case of hyperhemolytic syndrome(HHS)and explore the laboratory diagnostic method and clinical treatment strategy.Methods Serological tests such as blood typing,direct antiglobulin test(DAT),an-ti-screening and antibody identification were performed,The complete blood count(CBC),as well as hemolysis indicators such as lactate dehydrogenase(LDH)and bilirubin were tested,and the changes and progression of hemolysis were moni-tored.By using genotyping methods to test 32 common rare blood type antigens in clinical practice,the accuracy of antibody identification results was supported.Results The patient had anti-E antibodies and autoantibodies in her serum.After blood transfusion,LDH and bilirubin increased significantly,but hemoglobin dropped sharply,far lower than before transfusion,presenting with unique manifestations such as hemoglobinuria.The patient made a full recovery after treatment with high-dose intravenous immunoglobulin and corticosteroids.Conclusion HHS is a rare and potentially fatal diseases,which is mainly manifested by hemolysis and severe anemia,and the hemoglobin level after blood transfusion is often lower than that before blood transfusion.This patient had typical clinical manifestations of post-transfusion hyperhemolytic reaction.After timely and correct treatment,the patient recovered completely and was discharged,accumulating valuable clinical experience for the diagnosis and treatment of such disease.

hyperhemolytic syndrome(HHS)hemolytic anemiaautoantibodieshemoglobinuria

敖正才、朱文磊、肖明菊

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成都市第五人民医院 输血科,四川 成都 611130

超溶血综合征 溶血性贫血 自身抗体 血红蛋白尿

2022年度成都中医药大学"杏林学者"学科人才科研提升计划

YYZX202203

2024

中国输血杂志
中国输血协会 中国医学科学院输血研究所

中国输血杂志

CSTPCD
影响因子:1.279
ISSN:1004-549X
年,卷(期):2024.37(8)