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累及牙周组织的成人多灶性朗格汉斯细胞组织细胞增多症。

Adult multifocal Langerhan's cell histiocytosis involving periodontal tissues.

作者信息

Chava Vijay Kumar, Bhanu Vellampalli Udhaya

机构信息

Department of Periodontology, Narayana Dental College and Hospital, Nellore, Andhra Pradesh, India.

出版信息

J Indian Soc Periodontol. 2021 Mar-Apr;25(2):166-170. doi: 10.4103/jisp.jisp_22_20. Epub 2021 Mar 1.

Abstract

Langerhans cell histiocytosis (LCH) is a rare inflammatory neoplasia characterized by uncontrolled stimulation and abnormal proliferation of Langerhans cells (LCs). It can present as unifocal or multifocal with local and systemic manifestations which involves various internal organs and mucosal tissues. The clinical course varies among individuals, ranges from spontaneous resolution to life-threatening conditions with multisystem involvement. Prognosis relies on the involvement of risk organs (liver, spleen, and bone marrow) at diagnosis, mainly on organ dysfunction, and response to initial therapy. The diagnosis is based on histological study of tissues samples and shows tissue infiltration with LCs and the tumor cells immunopositive for s100/CD1a/Langerin/CD68 focal). In the oral cavity, LCH affects the alveolar or cortical bone and causes ulcerated lesions involving various mucosal and periodontal tissues. This article reports a case of adult multifocal LCH involving periodontal tissues with 1½ year posttreatment follow-up showing regression of the lesion.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的炎症性肿瘤,其特征为朗格汉斯细胞(LCs)不受控制的刺激和异常增殖。它可表现为单灶性或多灶性,伴有局部和全身表现,累及各种内脏器官和黏膜组织。临床病程因人而异,从自发缓解到危及生命的多系统受累情况不等。预后取决于诊断时风险器官(肝脏、脾脏和骨髓)的受累情况,主要取决于器官功能障碍以及对初始治疗的反应。诊断基于组织样本的组织学研究,显示有LCs组织浸润以及肿瘤细胞对s100/CD1a/朗格蛋白/CD68呈局灶性免疫阳性。在口腔中,LCH累及牙槽骨或皮质骨,并导致涉及各种黏膜和牙周组织的溃疡性病变。本文报告了一例累及牙周组织的成人多灶性LCH病例,治疗后随访1年半显示病变消退。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e05/8041086/8817bbc39e1b/JISP-25-166-g001.jpg

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