弥漫性肺淋巴管瘤病二例报告

Diffuse Pulmonary Lymphangiomatosis: Report of Two Cases

摘要: 目的：探讨2例弥漫性肺淋巴管瘤病(DPL)患者的临床特点。方法：对2018年和2019年青岛大学附属医院呼吸与危重症医学科收治的2例淋巴管瘤病的患者(例1经外科肺活检考虑为淋巴管瘤病，例2经核素淋巴显像证实)的临床资料进行回顾性分析。结果：例1 男，58岁，因“咳嗽、咳痰2月余”入院。患者行胸部增强CT示纵隔内广泛团块状略低密度影，外科肺活检病理结果提示：淋巴结呈反应性增生改变，另见多量大小不等的脉管，符合脉管瘤。例2 女，42岁，因“咳嗽、咳痰4月余”入院。患者行胸部增强CT示双侧锁骨上窝、纵隔内、腹膜后及腹腔弥漫性病变。后核素淋巴显像显示全身多发斑片、结节及肿块状软组织密度影，来源于淋巴系统。结论：DPL的CT表现多为双肺小叶间隔和支气管血管间质的光滑性增厚，乳糜性胸腔积液和胸膜增厚常见，也可见纵隔淋巴结肿大和纵隔脂肪密度增加。病理活检是诊断的金标准。

Abstract: Objective: To investigate the clinical features of 2 patients with diffuse pulmonary lymphangiomatosis. Methods: To explore the clinical data of 2 cases of diffuse lymphangiomatosis admitted to the respiratory and critical care medicine department of the Affiliated hospital of Qingdao university in 2018 and 2019 (case 1 was considered as lymphangiomatosis by surgical lung biopsy and case 2 was confirmed by radionuclide lymphography). Results: Case 1, male, 58 years old, was admitted to hospital due to “cough and expectoration for more than 2 months”. Enhanced chest CT of the patient showed extensive massive and slightly low-density shadows in the mediastinum. The pathological results of surgical lung biopsy indicated that: lymph nodes were found to show reactive hyperplasia and changes in the tissue, and a large number of vessels with different sizes were found to be consistent with angioma. Case 2, female, 42 years old, was admitted to hospital due to “cough and expectoration for more than 4 months”. Enhanced chest CT showed diffuse lesions in bilateral supraclavicular fossa, mediastinum, retroperitoneum and abdominal cavity. Nuclide imaging: systemic multiple patch, nodules, and mass of soft tissue density with different degree of imaging agent poly, consider possible sources of the lymphatic system. Conclusion: CT findings of DPL are mostly smooth thickening of bilateral pulmonary interlobular septum and bronchovascular interstitium, chylous pleural effusion and pleural thickening are common, mediastinal lymph node enlargement and mediastinal fat density can also be seen. Pathological biopsy is the gold standard of diagnosis.