摘要目的:探讨复杂性淋巴管异常的病理特征、临床表现及鉴别诊断。方法:收集2010年1月至2021年12月北京协和医院诊治的4例复杂性淋巴管异常患者临床及病理信息，并进行总结分析。结果:4例病例包括1例Gorham-Stout综合征及3例泛发性淋巴管异常，男女比为3∶1，年龄7~32岁。活检部位骨活检3例，支气管黏膜活检1例，镜下均可见弥漫分布的扩张或增生的淋巴管结构，Gorham-Stout综合征病例中可见明显的成骨现象。影像学上Gorham-Stout综合征可见骨皮质破坏，而泛发性淋巴管异常均为髓内溶骨性改变，3例泛发性淋巴管异常均合并凝血功能障碍，其中2例合并乳糜性浆膜腔积液。结论:复杂性淋巴管异常病理形态上以弥漫性分布的畸形淋巴管为特点，其诊断需结合临床及病理综合考虑。此类疾病治疗和预后上有所差别，因此需了解其临床及病理特点并进行鉴别诊断。

abstractsObjective:To investigate the clinicopathological features, clinical manifestations and different diagnosis of patients with complicated lymphatic anomaly.Methods:The clinical and pathologic data of four patients with complicated lymphatic anomaly diagnosed and treated in Peking Union Medical College Hospital from January 2000 to December 2021 were collected and analyzed.Results:One Gorham-Stout disease case and three generalized lymphatic anomaly cases were included in this cohort. Patients′ ages ranged from 7 to 32 years. There were three males and one female. The positions of biopsy included three bone biopsy and one bronchus biopsy. Microscopically, all cases showed diffuse enlarged lymphatic channels. At the same time, osteogenesis was obvious in Gorham-Stout disease case. Radiologically, cortical loss was seen in Gorham-Stout disease, and lytic bone confined to the medullary cavity presented in generalized lymphatic anomaly. The three generalized lymphatic anomaly cases also had coagulopathy, and two had effusion.Conclusions:The histologic feature of complicated lymphatic anomaly was diffuse lymphatic malformation, and the diagnosis depends on clinical and pathologic information. The treatment and prognosis of these diseases are different, and therefore it is necessary to understand their clinical and pathologic features and make the correct diagnosis.