Abstract
Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Adult LCH patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of other systems, resulting in patients being underdiagnosed and/or incompletely staged. Furthermore they may be treated with pediatric-based therapies which are less effective and sometimes more toxic for adults. The published literature on adult LCH cases lacks a comprehensive discussion on the differences between pediatric and adult patients and there are no recommendations for evaluation and comparative therapies. In order to fill this void, a number of experts in this field cooperated to develop the first recommendations for management of adult patients with LCH. Key questions were selected according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Based on the available literature up to December 2012, recommendations were established, drafts were commented by the entire group, and redrafted by the executive editor. The quality of evidence of the recommendations is predominantly attributed to the level of expert opinion. Final agreement was by consensus.
Highlights
Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body
Due to the diversity of clinical course of LCH, even recommendations which are established as standard of care may need to be critically appraised in an individual case and involvement of a LCH expert should be considered
A map of experts, reference centers and additional information about the disease is available on the website of Euro-Histio-Net and the Histiocytosis Association
Summary
Recommendation First step is smoking cessation in all patients Watchful waiting in a- or minor symptomatic patients Systemic steroid therapy in symptomatic patients Chemotherapy (e.g. 2-CDA) in progressive disease Consider lung transplantation in case of severe respiratory failure or major pulmonary hypertension. Test SS-LCH And No Disease Activity History (especially of thirst, polyuria, cough, dyspnea, bone pain, skin changes, neurological symptoms) Clinical assessment, blood count and blood chemistry (as described in baseline diagnostics), ultrasound. Chest XR After MS-LCH And With No Disease Activity History (especially of thirst, polyuria, cough, dyspnea, bone pain, skin changes, neurological symptoms) Clinical assessment, blood count and blood chemistry (as described in baseline diagnostics), ultrasound. Patients With pLCH History (in case of non-pulmonary symptoms: look for MS LCH, see Table 4) Diagnostic procedures are depending on symptoms und course of PLCH (baseline: Chest X-ray, lung function (+DCLO). Dermatologia, Fondazione IRCCS Ca Granda-Ospedale Maggiore Policlinico, Milano, Italy. 11Department of Internal Medicine, Hospital Saint Louis, Paris, France. 12Department of Radiotherapy and Radiation Oncology, Franziskus Hospital, Bielefeld, Germany. 13Radiation Oncology Center, Hamburg, Germany. 14Pulmonolgy Department, Saint Louis Teaching Hospital, Paris, France. 15Texas Children’s Cancer Center/Hematology Service, Houston, TX, USA
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