Doberauer Claus, Bornemann Christoph
Department of Internal Medicine, St. Franziskus-Hospital, Cologne, Germany.
Department of Internal Medicine, Protestant Clinics, Gelsenkirchen, Germany.
J Hematol. 2022 Aug;11(4):131-141. doi: 10.14740/jh1020. Epub 2022 Aug 30.
Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia belonging to the group of histiocytoses. Inflammatory tissue destruction with fibrosis can result in dysfunction in any organ. Our evaluation aimed to collect information on characteristics, courses, and therapeutic options of this rare disease pattern in adult patients with conclusions on prognostic factors and follow-up management.
The medical records of 194 adult patients with histologically confirmed LCH were evaluated in this retrospective study. Patients were treated at the Protestant Clinics in Gelsenkirchen from 2000 to 2014 and St. Franziskus-Hospital in Cologne until 2020.
The median age of onset was 38 years (18 to 79 years). In 65.5% of patients, only one organ was primarily involved, and in 34.5% of cases, multiple organs were involved. The skeleton, lungs, and skin were most commonly affected. In 15.5% of patients, pituitary insufficiency existed years before or at the time of diagnosis. The follow-up time of patients from the time of histologic diagnosis ranged from 6 to 408 months (median 49 months). Four patients died from sequelae of their underlying histiocytic disease. Irreversible late sequelae due to disease or therapy were detectable in 34% of patients. In 25.3% of the patients, the course of the disease could be controlled initially, but with the proviso of no smoking in case of lung involvement. Specific therapeutic measures such as surgery for solitary osteolysis, radiotherapy of osseous and cerebral manifestations, immunotherapy especially for lung and skin involvement, and chemotherapy for multisystem disease were primarily required in 74.7% of patients. As a result, 27.3% of all patients reached the nonactive stage. Of these, 26.4% had reactivation during the follow-up period. Of the remaining patients with continued active disease, 51.1% showed disease progression during follow-up.
Standardized diagnostics are required to capture the clinical picture. Due to the variable course, it is often sufficient to initially control with obligatory smoking cessation in case of pulmonary involvement. Follow-up examinations should be predominantly symptom-oriented with attention to possible late sequelae.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的炎症性髓系肿瘤，属于组织细胞增多症组。伴有纤维化的炎症组织破坏可导致任何器官功能障碍。我们的评估旨在收集成年患者中这种罕见疾病模式的特征、病程和治疗选择信息，并得出预后因素和随访管理的结论。
本回顾性研究评估了194例经组织学确诊为LCH的成年患者的病历。患者于2000年至2014年在盖尔森基兴的新教诊所接受治疗，直至2020年在科隆的圣弗朗西斯库斯医院接受治疗。
发病的中位年龄为38岁（18至79岁）。65.5%的患者仅一个器官首先受累，34.5%的病例多个器官受累。骨骼、肺部和皮肤最常受到影响。15.5%的患者在诊断前数年或诊断时存在垂体功能不全。患者从组织学诊断时起的随访时间为6至408个月（中位49个月）。4例患者死于潜在组织细胞疾病的后遗症。34%的患者可检测到因疾病或治疗导致的不可逆晚期后遗症。25.3%的患者疾病进程最初可以得到控制，但肺部受累时需戒烟。74.7%的患者主要需要采取特定的治疗措施，如对孤立性骨质溶解进行手术、对骨和脑部表现进行放疗、特别是对肺部和皮肤受累进行免疫治疗以及对多系统疾病进行化疗。结果，27.3%的所有患者达到非活动期。其中，26.4%在随访期间复发。在其余疾病持续活动的患者中，51.1%在随访期间出现疾病进展。
需要标准化诊断以掌握临床情况。由于病程多变，肺部受累时最初通过强制戒烟进行控制通常就足够了。随访检查应以症状为主，关注可能的晚期后遗症。
