gms | German Medical Science

Pulmonary Langerhanscell’ cell histiocytosis (PLCH) is a challenging disease to manage because of lack of well-defined prognostic indicators and limited understanding of the natural history of disease. Most adults with Langerhanscell’ cell histiocytosis (LCH) have pulmonary LCH and are smokers. In early stages, bilateral, micronodular or reticulonodular / interstitial infiltrates with sparing of the lung bases are typical findings. In the later stages, nodular lesions tend to be less frequent and cystic changes become more prominent. Most studies on LCH were performed retrospectively. Aim of the data base was to collect data of symptoms, diagnosis and course of the disease in recently diseased patients. In 67 adult patients (mean age 43 ± 7.7 years, 63% women, 37% men) data of history, diagnosis and course of the disease were assessed by questionnaire in patients with histologically proved LCH. Single organ involvement was found in 71% and 29 % multi organ disease. The most frequent organ involvement was those of the lungs (68%) followed by bones (43%) and skin (16%). Dyspnoea (69%) and cough (53%) were the most frequent symptoms. All patients with pulmonary single organ involvement were smokers or ex-smokers; however 28% of the patients with multisystem disease or nonpulmonary single system disease were non-smokers. The most frequent organ involvement of these patients was the lungs, but in 29 % of the patients multisystem disease was found. The data support the hypothesis of different clinical forms of adult LCH in smokers and non-smokers and that the LCH lesions in adults could be clonal as well as nonclonal, whereas in children the lesions are always clonal and the LC cells are less mature. A critical component of the management of all patients is smoking cessation. Although the exact effect of smoking cessation on the long-term outcome of PLCH has not been studied, smoking cessation may lead to stabilization of symptoms and even radiographic improvement in some patients. PLCH previously was considered a disease with a good prognosis, which has been challenged by recent data that clearly demonstrates those although many patients with PLCH have a good prognosis, a significant proportion of patients develop considerable morbidity and die prematurely from this disease. It is this subgroup of individuals with PLCH that need to be identified early in the course of disease and followed up carefully.