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http://dx.doi.org/10.1038/s41423-019-0302-5DOI Listing

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Current evidence suggests that ontogeny may account for the functional heterogeneity of some tissue macrophages, but not others. Here, we asked whether developmental origin drives different functions of skin Langerhans cells (LCs), an embryo-derived mononuclear phagocyte with features of both tissue macrophages and dendritic cells. Using time-course analyses, bone marrow chimeras, and fate tracing models, we found that the complete elimination of embryo-derived LCs at steady state results in their repopulation from circulating monocytes.

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Background: Langerhans cell histiocytosis (LCH) is a rare malignant disorder of epidermal antigen presenting cells. It is characterized by infiltration of various tissues with dendritic cells (Langerhans cells, LC) that express CD1a or CD207 (langerin), often leading to organ dysfunction. A patient with LCH required liver transplantation (LT) for LCH-associated biliary-tract disease.

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Indeterminate cell histiocytosis (ICH) is a rare histiocytic disorder characterized by a proliferation of CD1a and CD207/langerin cells. Recent molecular analyses have identified ETV3-NCOA2 translocation as a possible aetiopathogenesis of ICH. Herein, we describe the first Japanese case of ICH with ETV3-NCOA2 translocation.

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Langerhans cell histiocytosis in an infant.

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October 2024

Department of Gastroenterology and Pediatric Endoscopy Center, National Clinical Research Center for Child Health, National Children's Regional Medical Center, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.

A 2-month-old male infant presented with a persistent mild cough and a pink papule beneath the left eyelid. Imaging studies revealed diffuse lung opacities and a cystic shadow in the right middle lobe, with no evidence of bacterial or fungal infection. Skin biopsy demonstrated positive immunohistochemical staining for CD207/Langerin and CD1a, leading to a diagnosis of Langerhans-cell histiocytosis (LCH).

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Indeterminate DC histiocytosis is distinct from LCH and often associated with other hematopoietic neoplasms.

Blood Adv

November 2024

Department of Pathology, Ambroise-Paré Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), & EA4340-Biomarkers and clinical trials in Cancerology and Onco-Hematology, Versailles SQY University, Paris-Saclay University, Boulogne, France.

Article Synopsis
  • Indeterminate dendritic cell histiocytosis (IDCH) is a rare condition marked by a buildup of specific histiocytes, with particular immunophenotypic features and clinical presentations, mostly in older adults.
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