摘要
文章综述阵发性睡眠性血红蛋白尿症(PNH)常见并发症与合并症的管理策略,重点探讨血栓形成、慢性肾功能不全、肺动脉高压、骨髓衰竭及感染等常见并发症与合并症的处理。关键策略包括:以补体抑制剂为核心降低血栓及溶血风险,联合抗凝治疗预防血栓事件,通过支持治疗保护肾功能,靶向药物改善肺动脉高压;免疫抑制或造血干细胞移植纠正骨髓衰竭;强化疫苗接种及抗生素预防感染。文章还涵盖铁代谢异常、妊娠管理等特殊问题,并强调多学科协作的重要性。
This article reviews the management strategies for common complications and comorbidities of paroxysmal nocturnal hemoglobinuria(PNH),with a focus on addressing thrombosis,chronic renal insufficiency,pulmonary hypertension,bone marrow failure,and infections.Key strategies include:using complement inhibitors as the cornerstone to reduce thrombosis and hemolysis risks,combining anticoagulation therapy to prevent thrombotic events,protecting renal function through supportive care,and employing targeted medications to improve pulmonary hypertension.Immunosuppressive therapy or hematopoietic stem cell transplantation is utilized to correct bone marrow failure,while enhanced vaccination and antibiotic prophylaxis are emphasized to prevent infections.The article also covers special issues such as iron metabolism disorders and pregnancy management,highlighting the importance of multidisciplinary collaboration.
作者
贾晋松
JIA Jin-song(Department of Hematology,Peking University People's Hospital,Peking University Institute of Hematology,National Clinical Research Center for Hematologic Disease,Beijing 100044,China)
出处
《中国实用内科杂志》
北大核心
2025年第7期564-569,587,共7页
Chinese Journal of Practical Internal Medicine
基金
北京大学人民医院研究与发展基金(RDL2022-21)。
