Multisystem Langerhans cell histiocytosis in a pediatric patient: a rare case report with literature review and future directions

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Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of Langerhans cells that affects multiple organs. It presents variably in children, complicating diagnosis and treatment strategies. Understanding its genetic and clinical characteristics is crucial for its effective management. We report the case of a 13-year-old male with multisystem LCH involving the temporal bone, parotid gland, and lymph nodes, who presented with auricular pain and swelling. Despite the initial non-specific treatment, advanced imaging and biopsy confirmed the diagnosis. The patient underwent a treatment regimen according to the LCH4 protocol, which included Prednisolone and Vinblastine, and showed significant improvement. This case highlights the necessity of a multidisciplinary approach for diagnosing and managing LCH and illustrates the potential of genetic research and targeted therapies to improve outcomes. Future studies should explore the genetic basis of LCH and the potential links between immunization and disease onset, aiming to refine treatment protocols and enhance patient prognosis.

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Hanifa, H., Alshwayyat, S., Maksoud, C., Amro, A. M., Alshwayyat, M., Alshwayyat, T. A., … Ahmad, A. (2025). Multisystem Langerhans cell histiocytosis in a pediatric patient: a rare case report with literature review and future directions. Annals of Hematology, 104(5), 3067–3072. https://doi.org/10.1007/s00277-025-06375-1

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