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累及上颌骨和下颌骨的朗格汉斯细胞组织细胞增多症——病例报告

Langerhans Cell Histiocytosis Involving the Maxilla and Mandible - A Case Report.

作者信息

Sood Ramita V, Tiwari Ragini Ramendra, Weihsin Hu, Chauhan Anupama B, Trivedi Nisarg R, Gadhiya Bansarim B

机构信息

Department of Oral and Maxillofacial Surgery, Ahmedabad Municipal Corporation Dental College and Hospital, Ahmedabad, Gujarat, India.

出版信息

Ann Maxillofac Surg. 2025 Jan-Jun;15(1):102-105. doi: 10.4103/ams.ams_136_24. Epub 2025 May 9.

Abstract

RATIONALE

Langerhans cell histiocytosis (LCH) is a rare disease involving neoplastic proliferation of myeloid precursor cells, primarily affecting bones such as ribs, pelvis, femur, and mandible with rare simultaneous involvement of the maxilla and mandible.

PATIENT CONCERNS AND DIAGNOSIS

A 48-year-old male presented with swelling and tooth mobility since 6 months. Orthopantomogram revealed extensive destruction in the mandible and right maxillary posterior region. Histological examination confirmed LCH using immunohistochemical staining with S-100, CD1a.

TREATMENT

As patient refused of surgery, intralesional methylprednisolone injections were selected as non-invasive treatment approach for localized LCH.

OUTCOME

Three months post-treatment, remission was observed, though some residual lesion remained. Follow-up at six months showed no further disease progression, supporting the efficacy of corticosteroid injections.

TAKE-AWAY LESSONS: Treatment should be based on the diagnosis of LCH along with the extent of involvement of bones. Based on review of the literature and our own findings, intralesional corticosteroids were found to be effective for localized LCH.

摘要

理论依据

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见疾病,涉及髓样前体细胞的肿瘤性增殖,主要影响肋骨、骨盆、股骨和下颌骨等骨骼,上颌骨和下颌骨同时受累的情况罕见。

患者情况与诊断

一名48岁男性自6个月以来出现肿胀和牙齿松动。全景曲面断层片显示下颌骨和右上颌后牙区广泛破坏。组织学检查通过S-100、CD1a免疫组化染色确诊为LCH。

治疗

由于患者拒绝手术,选择病灶内注射甲基强的松龙作为局限性LCH的非侵入性治疗方法。

结果

治疗3个月后,观察到病情缓解,尽管仍有一些残留病灶。6个月的随访显示无进一步疾病进展,支持皮质类固醇注射的疗效。

经验教训

治疗应基于LCH的诊断以及骨骼受累程度。基于文献回顾和我们自己的发现,病灶内注射皮质类固醇对局限性LCH有效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d670/12321178/ccbe67e90a93/AMS-15-102-g001.jpg

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