Barclay Mhairi, Devaney Rebecca, Bhatt Jayesh M
Paediatric Respiratory Medicine, Nottingham University Hospitals NHS Trust, Nottingham, UK.
Nottingham Children's Hospital, Nottingham University Hospitals NHS Trust, Nottingham, UK.
Breathe (Sheff). 2020 Jun;16(2):200003. doi: 10.1183/20734735.0003-2020.
Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease. Unlike pulmonary Langerhans cell histiocytosis (LCH) in adults, which is often seen as an isolated condition with smoking being a major risk factor, isolated pPLCH is vanishingly rare in children and it is most often a component of multisystem LCH. Diagnosis should be based on histological and immunophenotypic examination of affected tissue in addition to clinical and radiological features. It should be considered an important differential for diffuse cystic lung disease in paediatric patients. Recent progress in the biological understanding of the disease supports the classification of LCH as an inflammatory myeloid neoplasia. Chemotherapy and specific management of respiratory complications are the mainstays of treatment. The lungs are no longer considered a "risk organ" in LCH as pulmonary involvement is not associated with a worse prognosis than the involvement of other organs. Multidisciplinary treatment approaches are needed. Prognosis can be good but is adversely influenced by multisystem involvement, and complications such as pneumothoraces and respiratory failure can be life threatening. This review aims to give an overview of this condition, with a focus on the diagnosis, monitoring and management of complications such as pneumothoraces and respiratory failure, which can be challenging for the paediatric respiratory specialist.
To give an overview of paediatric pulmonary LCH.To discuss the differential diagnosis of paediatric cystic lung disease.
儿童肺朗格汉斯细胞组织细胞增多症（pPLCH）是一种罕见的弥漫性囊性肺疾病。与成人肺朗格汉斯细胞组织细胞增多症（LCH）不同，成人LCH常表现为孤立性疾病，吸烟是主要危险因素，而孤立性pPLCH在儿童中极为罕见，它最常是多系统LCH的一个组成部分。诊断应基于对受累组织的组织学和免疫表型检查，以及临床和放射学特征。在儿科患者中，应将其视为弥漫性囊性肺疾病的重要鉴别诊断。对该疾病生物学认识的最新进展支持将LCH归类为炎症性髓系肿瘤。化疗和呼吸并发症的特异性管理是主要治疗方法。在LCH中，肺不再被视为“风险器官”，因为肺部受累与其他器官受累相比，预后并不更差。需要多学科治疗方法。预后可能良好，但多系统受累会对其产生不利影响，气胸和呼吸衰竭等并发症可能危及生命。本综述旨在概述这种疾病，重点关注气胸和呼吸衰竭等并发症的诊断、监测和管理，这些对儿科呼吸专科医生来说可能具有挑战性。
概述儿童肺LCH。讨论儿童囊性肺疾病的鉴别诊断。
