CT诊断肺朗格汉斯细胞组织细胞增生症

CT in diagnosis of pulmonary Langerhans cell histiocytosis

目的探讨肺朗格汉斯细胞组织细胞增生症(PLCH)的CT表现。方法回顾性分析10例经病理证实PLCH患者的临床资料及CT影像资料,分析病变的分布、大小和形态等CT特征。结果单系统(SS)朗格汉斯细胞组织细胞增生症(LCH)7例,其中早期1例,中晚期6例;多系统(MS)LCH 3例,其中早期1例,中晚期2例。10例病变均呈弥漫、对称性分布,并以中上肺野分布为主。2例早期PLCH患者CT表现以多发小叶中央性结节为主,并见厚壁或薄壁空洞结节;8例中晚期患者,CT表现以囊腔为主,为圆形或类圆形囊腔、形态多样的融合囊腔合并/不合并少量结节、空洞结节,其中2例融合性囊腔形成肺大疱,并破裂产生气胸。结论双侧中上肺野多发小叶中心性分布结节、空洞结节,合并囊腔,特别是形态多样的囊腔为PLCH的CT特征表现。

Objective To investigate the CT features of pulmonary Langerhans cell histiocytosis （LCH）. Methods Clini- cal and CT features of 10 patients with pulmonary LCH proved by biopsy pathology were retrospectively analyzed. The CT features including distribution, size and contour of the lesions were analyzed. Results Seven patients were single system （SS） pulmonary LCH, including 1 case of early stage and 6 cases of mid-late stage; 3 cases were multiple systems （MS） LCH, including 1 case of early stage and 2 cases of mid-late stage. All the cases presented diffused distribution and pre- dominately in upper and middle lung field. In 2 cases of early stage, CT features were characterized by multiple bronchiolo- centric nodules. Some nodules could present faint lucent centre or be mostly cavitary. In 8 cases of mid-late stage, cysts predominated on nodules, which appeared round or bizarre, 2 cases of them showed cyst fusion forms bulla and pneumotho- rax. Conclusion CT features of pulmonary LCH are quite specific, including diffuse distribution of bronchiolocentric nod- ules in bilateral upper-middle lung, with cavitary nodes, even cysts, especially bizarre-shaped cysts.