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Vol. 161. Issue 4.
Pages 166-175 (August 2023)
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Vol. 161. Issue 4.
Pages 166-175 (August 2023)
Review
Histiocytosis
Histiocitosis
Visits
18
Gemma Rocamora-Blancha,b,
Corresponding author
grocamora@bellvitgehospital.cat

Corresponding author.
, Fina Climentb,c, Xavier Solanicha,b
a Servicio de Medicina Interna, Hospital Universitari de Bellvitge, L’Hospitalet de Llobregat, Barcelona, Spain
b Instituto de Investigación Biomédica de Bellvitge (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain
c Servicio de Anatomía Patológica, Hospital Universitari de Bellvitge, L’Hospitalet de Llobregat, Barcelona, Spain
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Tables (3)
Table 1. Classification of histiocytoses. Langerhans cell-related (group L), cutaneous and mucocutaneous (group C), malignant histiocytosis (group M), Rosai-Dorfman disease (group R), haemophagocytic lymphohistiocytosis and macrophage activation syndrome (group H).
Table 2. Pathological, immunohistochemical and molecular features of Langerhans cell histiocytosis, Erdheim-Chester disease and Rosai-Dorfman diseasea.
Table 3. Treatment regimen in Langerhans cell histiocytosis, Erdheim-Chester disease and Rosai-Dorfman disease.
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Abstract

Histiocytosis 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.

Keywords:
Histiocytosis
Langerhans
Erdheim-Chester
Rosai-Dorfman
BRAF V600E
Resumen

Las histiocitosis son un grupo de enfermedades raras que se caracterizan por la inflamación y acúmulo de células derivadas de los monocitos y macrófagos en diferentes tejidos. La clínica es muy variable, desde formas leves con afectación de un solo órgano a formas multisistémicas graves que pueden comprometer la vida. Su diagnóstico se basa en la clínica, hallazgos radiológicos y la anatomía patológica. Se recomienda realizar una biopsia del tejido afecto en todos los casos dado que puede tener implicaciones terapéuticas. En este sentido, durante la última década se ha identificado mutaciones en tejido afecto que condicionan activación de la vía de las proteínas cinasas activadas por mitógenos (MAPK/ERK) y fosfatidilinositol 3 kinasa (PI3K/AKT), en proporción variable, en función del tipo de histiocitosis. En esta revisión nos centramos fundamentalmente en la histiocitosis de Células de Langerhans, la Enfermedad de Erdheim-Chester y la Enfermedad de Rosai-Dorfman.

Palabras clave:
Histiocitosis
Langerhans
Erdheim-Chester
Rosai-Dorfman
BRAF V600E

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