U.O. di Pneumologia e Terapia Semi-Intensiva, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare Ospedale San Giuseppe MultiMedica, via San Vittore 12, 20123 Milano, Italy.
U.O. di Pneumologia e Terapia Semi-Intensiva, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare Ospedale San Giuseppe MultiMedica, via San Vittore 12, 20123 Milano, Italy.
Eur J Intern Med. 2015 Jun;26(5):351-6. doi: 10.1016/j.ejim.2015.04.001. Epub 2015 Apr 17.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial disease affecting primarily young adult smokers. In order to highlight the clinical features of the disease, we conducted a retrospective analysis on clinical data of PLCH patients followed at our center; moreover, we reviewed the current literature on PLCH.
Between January 2004 and July 2014, 40 patients with PLCH were evaluated at our Division. The average patients' age was 40 (± 14) years, and 22 of them were females. Diagnosis was based on search of CD1a+ cells in the bronchoalveolar lavage (10 patients), lung biopsy (8 patients), or cystic bone lesion's biopsy (2 patients); in 12 patients, diagnosis was achieved on the basis of the clinical-radiological data. The principal manifestation of PLCH was the presence of cysts involving upper lung zones with costophrenic sparing on chest CT scan (in 25 patients); micronodular pattern in the middle-upper zone and combination of the two radiological patterns were less frequently observed (in 9 and 6 patients, respectively). Pulmonary hypertension was found in 4 patients. Extra pulmonary manifestations were diabetes insipidus, bone lesions, and skin involvement (in 5, 7, and 1 patient, respectively). For 25 patients, smoking cessation was the only required therapy. Treatments with low dose of prednisolone, vinblastine and prednisolone, or 6-mercaptopurin were reserved for patients with major pulmonary or extra-pulmonary involvement (for 11, 4, and 5 patients, respectively). In conclusion, PLCH is a rare, multi-systemic disease; early diagnosis, accurate staging and smoking cessation are considered critical in PLCH management.
肺朗格汉斯细胞组织细胞增生症（PLCH）是一种罕见的间质性疾病，主要影响年轻成年吸烟者。为了突出该疾病的临床特征，我们对在我中心接受治疗的 PLCH 患者的临床数据进行了回顾性分析；此外，我们还回顾了当前关于 PLCH 的文献。
2004 年 1 月至 2014 年 7 月，我科共评估了 40 例 PLCH 患者。患者平均年龄为 40（±14）岁，其中 22 例为女性。诊断依据为支气管肺泡灌洗液中 CD1a+细胞（10 例）、肺活检（8 例）或囊性骨病变活检（2 例）中寻找朗格汉斯细胞；12 例患者根据临床-影像学数据进行诊断。PLCH 的主要表现为胸部 CT 扫描上肺上区存在含气囊肿，伴肋膈角保留（25 例）；中-上区微结节模式和两种影像学模式的组合较少见（分别为 9 例和 6 例）。4 例患者发现肺动脉高压。肺外表现为尿崩症、骨病变和皮肤受累（分别为 5、7 和 1 例）。25 例患者仅需戒烟。对于有严重肺外或肺部受累的患者，采用小剂量泼尼松龙、长春碱和泼尼松龙或 6-巯基嘌呤治疗（分别为 11、4 和 5 例）。总之，PLCH 是一种罕见的多系统疾病；早期诊断、准确分期和戒烟被认为是 PLCH 管理的关键。
