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一名儿科患者的多系统朗格汉斯细胞组织细胞增多症:一例罕见病例报告及文献综述与未来方向

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

作者信息

Hanifa Hamdah, Alshwayyat Sakhr, Maksoud Carolin, Amro Alhareth M, Alshwayyat Mustafa, Alshwayyat Tala Abdulsalam, Alkhatib Mesk, Alsaleh Basil, Odat Ramez M, Ahmad Ammar

机构信息

Faculty of Medicine, University of Kalamoon, Al-Nabk, Syria.

Research Associate, King Hussein Cancer Center, Amman, Jordan.

出版信息

Ann Hematol. 2025 May;104(5):3067-3072. doi: 10.1007/s00277-025-06375-1. Epub 2025 Apr 23.

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.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见疾病,其特征为朗格汉斯细胞增殖,可累及多个器官。它在儿童中的表现多样,使诊断和治疗策略变得复杂。了解其遗传和临床特征对有效管理该病至关重要。我们报告一例13岁男性多系统LCH病例,病变累及颞骨、腮腺和淋巴结,患者表现为耳部疼痛和肿胀。尽管最初进行了非特异性治疗,但进一步的影像学检查和活检确诊了该病。该患者按照LCH4方案接受了治疗,包括泼尼松龙和长春碱,病情有显著改善。该病例凸显了多学科方法在LCH诊断和管理中的必要性,并说明了基因研究和靶向治疗改善治疗效果的潜力。未来的研究应探索LCH的遗传基础以及免疫接种与疾病发病之间的潜在联系,旨在完善治疗方案并改善患者预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c2a/12141398/27e3e86c2be2/277_2025_6375_Fig1_HTML.jpg

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