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Histiocytoses

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The Microbiome in Rheumatic Diseases and Infection

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Abstract

Histiocytoses are rare, often systemic diseases hallmarked by tissue infiltration by abnormal histiocytes bearing peculiar morphological and immunohistochemical characteristics. They are classified based on cell type (e.g. Langerhans cell histiocytosis (LCH) and non-Langerhans forms such as Erdheim-Chester disease (ECD)), preferential organ involvement (e.g. cutaneous forms) and clinical aggressiveness (e.g. malignant histiocytoses). Some histiocytoses are clearly clonal disorders, often driven by somatic mutations of proto-oncogenes, whereas others can be reactive to or associated with systemic immune-mediated or infectious diseases. This chapter will mainly focus on primary systemic histiocytoses such as LCH and ECD and will discuss the role of infections in these complex diseases.

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Abbreviations

CNS:

Central nervous system

CTLs:

Cytotoxic T cells

ECD:

Erdheim-Chester disease

HHV6:

Human herpesvirus 6

HIV:

Human immunodeficiency virus

HLH:

Haemophagocytic lymphohistiocytosis

IFN-y:

Interferon-y

IFNα:

Interferon-α

IL-12:

Interleukin-12

LCH:

Langerhans cell histiocytosis

MRI:

Magnetic resonance imaging

mTOR:

Mammalian target of rapamycin

NK:

Natural killer

PET:

Positron emission tomography

RDD:

Rosai-Dorfman disease

Th1:

T-helper 1

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Authors and Affiliations

  1. Nephrology Unit, University Hospital, Parma, Italy

    Augusto Vaglio & Rossana Rocco

  2. Department of Internal Medicine, French Reference Center for Rare Auto-immune and Systemic Diseases, Institut E3M, AP-HP, Pitié-Salpêtrière Hospital, Paris, France

    Julien Haroche

  3. Université Pierre et Marie Curie, University Paris 6, Paris, France

    Julien Haroche

  4. EA4340 and Pathology Department, Versailles University and Ambroise Paré Hospital, Boulogne, France

    Jean-François Emile

Authors
  1. Augusto Vaglio
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  2. Rossana Rocco
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  3. Julien Haroche
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  4. Jean-François Emile
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Corresponding author

Correspondence to Augusto Vaglio .

Editor information

Editors and Affiliations

  1. Faculty of Medicine, Cairo University, Cairo, Egypt

    Gaafar Ragab

  2. University of Alabama, Birmingham, Alabama, USA

    T. Prescott Atkinson

  3. University of Alabama, Birmingham, Alabama, USA

    Matthew L. Stoll

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Vaglio, A., Rocco, R., Haroche, J., Emile, JF. (2018). Histiocytoses. In: Ragab, G., Atkinson, T., Stoll, M. (eds) The Microbiome in Rheumatic Diseases and Infection. Springer, Cham. https://doi.org/10.1007/978-3-319-79026-8_29

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  • DOI: https://doi.org/10.1007/978-3-319-79026-8_29

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  • Online ISBN: 978-3-319-79026-8

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