Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net
Por:
Girschikofsky, M, Arico, M, Castillo, D, Chu, A, Doberauer, C, Fichter, J, Haroche, J, Kaltsas, GA, Makras, P, Marzano, AV, de Menthon, M, Micke, O, Passoni, E, Seegenschmiedt, HM, Tazi, A, McClain, KL
Publicada:
14 may 2013
Resumen:
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.
Filiaciones:
Girschikofsky, M:
Elisabethinen Hosp, Ctr Hematol Stem Cell Transplantat Hemostasis & M, Dept Med 1, A-4010 Linz, Austria
Arico, M:
Azienda Osped Univ A Meyer, Dept Pediat Hematol Oncol, Florence, Italy
Castillo, D:
Hosp Santa Creu & Sant Pau, Dept Resp Med, Barcelona, Spain
Chu, A:
Imperial NHS Trust, London, England
Doberauer, C:
Protestant Clin, Clin Internal Med, Gelsenkirchen, Germany
Fichter, J:
Paracelsus Klin, Osnabruck, Germany
Haroche, J:
Grp Hosp Pitie Salpetriere, Serv Med Interne, F-75634 Paris, France
Kaltsas, GA:
Univ Athens, Sch Med, Dept Pathophysiol, GR-11527 Athens, Greece
Makras, P:
251 Hellenic Air Force & VA Gen Hosp, Dept Endocrinol & Diabet, Athens, Greece
Marzano, AV:
Fdn IRCCS Ca Granda Osped Maggiore Policlin, UO Dermatol, Milan, Italy
de Menthon, M:
Hosp St Louis, Dept Internal Med, Paris, France
Micke, O:
Franziskus Hosp, Dept Radiotherapy & Radiat Oncol, Bielefeld, Germany
Passoni, E:
Fdn IRCCS Ca Granda Osped Maggiore Policlin, UO Dermatol, Milan, Italy
Seegenschmiedt, HM:
Radiat Oncol Ctr, Hamburg, Germany
Tazi, A:
St Louis Teaching Hosp, Dept Pulmonol, Paris, France
McClain, KL:
Hematol Serv, Texas Childrens Canc Ctr, Houston, TX USA
Gold, Green Published
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