Pathophysiology and treatment of adult Langerhans cell histiocytosis

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  • TOJO Arinobu
    Division of Molecular Therapy, Advanced Clinical Research Center, The Institute of Medical Science, The University of Tokyo
  • KOBAYASHI Masayuki
    Division of Molecular Therapy, Advanced Clinical Research Center, The Institute of Medical Science, The University of Tokyo

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Other Title
  • ランゲルハンス細胞組織球症の基礎と臨床
  • ランゲルハンス サイボウ ソシキキュウショウ ノ キソ ト リンショウ

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<p>Langerhans cell histiocytosis (LCH) is a rare disease characterized by tissue infiltration of clusters of CD1a+/CD207+ histiocyte-like cells and a resultant surrounding inflammatory reaction. Because of its morphological similarity to cutaneous Langerhans cells, LCH was formerly named histiocytosis X in 1987. However, its cell lineage appears closely related to myeloid dendritic cells. In 2010, BRAF-V600E was detected in biopsy specimens from the majority of LCH patients. The subsequent observation of extracellular signal-regulated kinase phosphorylation in almost all LCH samples suggested that LCH was a neoplasm provoked by activation of the mitogen-activated protein (MAP) kinase pathway. Therefore, the 2016 Revised Classification by the Histiocyte Society defined LCH as an inflammatory myeloid neoplasm. Although a series of global and domestic clinical trials have improved the prognosis of pediatric LCH patients, no standard therapy with a high level of evidence for adult cases exists. Generally, LCH patients have a favorable prognosis, but delayed diagnosis and intervention may cause severe damage to the involved organs, resulting in a poor quality of life. Here we present recent advances in the pathophysiology and treatment of LCH to enlighten the understanding of this orphan disease.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 61 (9), 1028-1034, 2020

    The Japanese Society of Hematology

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