[Reference guide for the clinical management of adult Langerhans cell histiocytosis (key points from the first edition, 2025)].

Authors

Aki Sato

Published in

[Rinsho ketsueki] The Japanese journal of clinical hematology. Volume 67. Issue 9. Pages 1194-1202.

Abstract

According to the registry of the Japanese Society of Hematology, adult histiocytic neoplasms are extremely rare, with approximately 40 cases reported annually, and the most common among them is Langerhans cell histiocytosis (LCH). Lesions may involve multiple organs, including bone, skin, lung, the central nervous system, and the endocrine system. Without a hematology perspective, the neoplastic nature of LCH may not be apparent, resulting in missed diagnoses. Central diabetes insipidus, anterior pituitary dysfunction, and LCH-associated neurodegeneration, once developed, often result in irreversible impairment. Because delayed therapeutic intervention can result in permanent organ damage, it is essential for hematologists to understand the "minimum initial management" required to protect patient outcomes. This review summarizes the key points of diagnosis and initial management in adult LCH based on the first edition of the Adult LCH Clinical Reference Guide (2025).

PMID:
42951634
Bibliographic data and abstract were imported from PubMed on 29 Oct 2026.

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