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Adult Langerhans Cell Histiocytosis

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Histiocytic Disorders

Abstract

Most of the knowledge regarding the diagnosis and treatment of adult Langerhans cell histiocytosis (LCH) patients is derived from pediatric studies. Adult LCH patients are often evaluated by physicians who focus only on the most obviously affected organ without sufficient evaluation of other systems, which may lead to an erroneous diagnosis in some patients. In addition, treatment with pediatric-based regimens, often referred to as standard therapy, seems to be less effective and sometimes more toxic in adults.

The published literature on adult LCH lacks a comprehensive discussion on the differences between pediatric and adult patients, and it is still debatable what and how much can be extrapolated from the younger age cohort. Isolated primary pulmonary LCH, in almost all cases associated with smoking, is a common presenting pattern in adults and has particular clinico-radiographic and treatment features that warrant its characterization as a separate entity.

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Authors and Affiliations

  1. Internal Medicine I, (Hemostasis, Hematology and Stem Cell Transplantation and Medical Oncology), Ordensklinikum Linz – Elisabethinen Hospital, Fadingerstraße 1, 4020, Linz, Austria

    Michael Girschikofsky MD

  2. Assistance Publique-Hôpitaux de Paris, Saint-Louis Hospital, National Reference Center for Langerhans Cell Histiocytosis, Pulmonary Department, Paris, France

    Abdellatif Tazi MD, PhD

  3. University Paris Diderot, Sorbonne Paris Cité, Inserm UMR-1153 (CRESS), Biostatistics and Clinical Epidemiology Research Team (ECSTRA), Paris, France

    Abdellatif Tazi MD, PhD

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  1. Michael Girschikofsky MD
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Correspondence to Michael Girschikofsky MD .

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  1. University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada

    Oussama Abla

  2. Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Hamburg, Germany

    Gritta Janka

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Girschikofsky, M., Tazi, A. (2018). Adult Langerhans Cell Histiocytosis. In: Abla, O., Janka, G. (eds) Histiocytic Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-59632-7_7

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