朗格汉斯细胞组织细胞增生症的分型及影像学表现

Classification and Imaging Manifestations of Langerhans Cell Histiocytosis

目的 探讨朗格汉斯细胞组织细胞增生症(LCH)的分型及其影像学表现,提高对该病的认识及诊断水平.方法 回顾性分析经病理确诊的9例LCH的临床资料、影像资料及病理表现.结果 9例LCH中8例为单系统LCH(骨骼系统7例、单纯肺受累1例),1例为多系统LCH.骨骼受累多表现为"穿凿样"骨质破坏,肺受累表现为双肺多发大小不等、不规则囊样病变及小结节影,并合并左侧气胸.1例多系统LCH累及骨骼系统、肺及垂体,垂体MRI表现为T_(1)WI垂体后叶高信号消失,垂体柄增粗、明显强化.结论 通过影像学表现对LCH进行分型,结合实验室检查,可提高该病的早期诊断,对患者进行及时治疗并评估疗效.

Objective To evaluate the classification of Langerhans cell histiocytosis(LCH)and its imaging findings,and improve the understanding and diagnosis of this disease.Methods The clinical data,imaging data and pathological manifestations of 9 cases of LCH diagnosed by pathology were retrospectively analyzed.Results Of the 9 cases of LCH,8 cases were single-system LCH(7 cases of skeletal system,1 case of pure lung involvement),and 1 case was multi-system LCH.Bone involvement was mostly manifested as perforating bone destruction,and lung involvement was manifested as multiple irregular cystic lesions and small nodules of varying sizes in both lungs,combined with left pneumothorax.One case of multisystem LCH involved the skeletal system,lung and pituitary.MRI of the pituitary showed that the posterior pituitary hyperintensity disappeared on T_(1)WI,and the pituitary stalk was thickened and strengthened.Conclusion Classification of LCH through imaging findings,combined with laboratory examinations,can improve the early diagnosis accuracy of the disease,timely treatment of patients and evaluation of the efficacy.