| 管鑫,吴家良,朱远航.淋巴管肌瘤病 201例临床特点及中西医结合治疗疗效分析[J].安徽医药,2025,29(5):935-940. |
| 淋巴管肌瘤病 201例临床特点及中西医结合治疗疗效分析 |
| Clinical features and effect of integrated traditional Chinese and Western medicine in the treatment of lymphangioleiomyomatosis:an analysis of 201 cases |
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| DOI：10.3969/j.issn.1009-6469.2025.05.016 |
| 中文关键词: 淋巴管肌瘤病 罕见病 西罗莫司 四逆散 |
| 英文关键词: Lymphangioleiomyomatosis Rare disease Sirolimus Sini San |
| 基金项目:上海市进一步加快中医药传承创新发展三年行动计划［ZY(2021-2023)-0207-02］；上海市卫健委中医药科研项目( 2024QN091)；上海市黄浦区科委科研项目( HZQ202110) |
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| 中文摘要: |
| 目的总结淋巴管肌瘤病( LAM)的临床特点及诊疗情况，提高中西医结合治疗 LAM疗效，减少漏诊和误诊。方法收集 2012年 1月至 2021年 12月于上海市黄浦区香山中医医院 LAM中医特色专科门诊就诊并定期随访的 201例 LAM病人的临床诊治资料，进行分析总结。结果 201例 LAM病人均为女性，确诊年龄( 36.18±8.73)岁，因疾病罕见，误诊率高达 39.80%，最常见的误诊疾病为气胸。诊断标准近年发生改变，在诊断过程中正在逐渐减少使用有创操作。与普通人群相比，患有 LAM的女性生育能力较差，生育的孩子也较少，妊娠发生早产和流产的数量更多，晚期 LAM病人应避免受孕。临床表现最常发生为气胸( 48.76%)、肾血管平滑肌脂肪瘤( 33.33%)、结节性硬化( 7.96%)、乳糜胸( 22.40%)、乳糜腹( 9.95%)、腹膜后包块( 27.9%)、肺动脉高压( 18.41%)。存在肺功能损害、气胸和乳糜胸的病人建议服用西罗莫司，上海市黄浦区香山中医医院 LAM专病团队在总结经验的基础上，制定了淋巴管肌瘤病中医诊疗方案，认为 LAM总的治疗原则为疏肝理气、补肾敛肺，主要方药为四逆散加减，根据 LAM病人不同证型进行辨证施治用药，服用中药后可以明显改善气促、咳喘、胸闷等临床症状。结论 LAM属于我国发布的罕见病之一，是一种以肺部弥漫性囊性病变为特征的多系统转移性肿瘤疾病，病人的症状取决于受累的器官。因为发病率极低、临床诊断困难、对疾病认识程度不足等原因， LAM的误诊率、漏诊率较高。中医药四逆散加减治疗 LAM具有独特优势。 |
| 英文摘要: |
| Objective To summarize the clinical features, diagnosis and treatment of lymphangioleiomyomatosis (LAM), to improvethe curative effect of integrated traditional Chinese and Western medicine in the treatment of LAM, and to reduce missed and misdiag-nosed cases.Methods We collected and analyzed the clinical diagnosis and treatment data of 201 LAM patients who were treated inthe LAM TCM Specialty Clinic of Xiangshan Hospital of Traditional Chinese Medicine, Huangpu District, Shanghai from January 2012to December 2021 and followed up regularly.Results The 201 LAM patients were all female, with an average diagnosis age of (36.18±8.73) years, and the misdiagnosis rate was as high as 39.8% due to rareness of the disease, and LAM was most commonly misdiagnosedas pneumothorax. The diagnostic criteria of the disease have changed in recent years, and the use of invasive procedures in the diagnos-tic process is gradually decreasing. Compared with the general population, women with LAM are less fertile, have fewer children, andhave a higher rate of premature birth and miscarriages, and thus pregnancy should be avoided in patients with advanced LAM. Themost common clinical manifestations were pneumothorax (48.76%), renal angiomyolipoma (33.33%), tuberous sclerosis (7.96%), chylo-thorax (22.40%), chyloperitoneum (9.95%), retroperitoneal mass (27.9%), pulmonary hypertension (18.41%). Patients with lung func-tion impairment, pneumothorax and chylothorax were recommended to take sirolimus. On the basis of summarizing experience, theLAM disease specialist team of Xiangshan Hospital of Traditional Chinese Medicine formulated a TCM diagnosis and treatment plan forlymphangioleiomyomatosis, and believed that the overall treatment principle of LAM disease was to invigorate the kidney and restrainthe lung, soothe the liver and regulate qi. The main prescription was Sini San. The dosage of medicine was determined according to thesyndrome differentiation of LAM patients. After taking the traditional Chinese medicine, clinical symptoms of shortness of breath,cough and asthma, chest tightness, etc. can be significantly improved.Conclusions LAM is one of the rare diseases listed by China, characterized by diffuse cystic lung lesions, and is a multi-systemic metastatic tumor disease. The symptoms of patients depend on the organs affected. Due to its extremely low incidence rate, difficulty in clinical diagnosis, and insufficient understanding of the disease,the misdiagnosis and missed diagnosis rates for LAM are relatively high. Traditional Chinese Medicine, specifically the Sini San formu-la with modifications, has unique advantages in treating LAM. |
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