• Medicina clinica · Aug 2023

    Review

    Histiocytosis.

    • Gemma Rocamora-Blanch, Fina Climent, and Xavier Solanich.
    • Servicio de Medicina Interna, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, España; Instituto de Investigación Biomédica de Bellvitge (IDIBELL), L'Hospitalet de Llobregat, Barcelona, España. Electronic address: grocamora@bellvitgehospital.cat.
    • Med Clin (Barc). 2023 Aug 25; 161 (4): 166175166-175.

    AbstractHistiocytosis is a group of rare diseases characterized by inflammation and accumulation of cells derived from monocytes and macrophages in different tissues. The symptoms are highly variable, from mild forms with involvement of a single organ to severe multisystem forms that can be life compromising. The diagnosis of histiocytosis is based on the clinic, radiological findings and pathological anatomy. A biopsy of the affected tissue is recommended in all cases as it may have therapeutic implications. During the last decade, some mutations have been identified in the affected tissue that condition activation of the MAPK/ERK and PI3K/AKT pathway, in a variable proportion depending on the type of histiocytosis. In this review we mainly focus on Langerhans Cell Histiocytosis, Erdheim-Chester Disease and Rosai-Dorfman Disease.Copyright © 2023 Elsevier España, S.L.U. All rights reserved.

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