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一名累及口腔的成年朗格汉斯细胞组织细胞增多症:带来诊断挑战

Langerhans Cell Histiocytosis in an Adult with Oral Cavity Involvement: Posing a Diagnostic Challenge.

作者信息

Nangalia Richa, Chatterjee Rudra Prasad, Kundu Sanchita, Pal Mousumi

机构信息

Department of Oral and Maxillofacial Pathology, Guru Nanak Institute of Dental Sciences and Research, Kolkata, West Bengal, India.

Department of Oral and Dental Sciences, JIS University, Kolkata, West Bengal, India.

出版信息

Contemp Clin Dent. 2019 Jan-Mar;10(1):154-157. doi: 10.4103/ccd.ccd_432_18.

Abstract

Langerhans cell histiocytosis (LCH) is a rare hematological disorder characterized by local or generalized, uncontrolled proliferation and infiltration of Langerhans type of histiocytic cells. It represents a spectrum of clinicopathologic disorders, ranging from a highly aggressive and frequently fatal multisystem disease to an easily cured solitary lesion of bone. Involvement of children and the younger age group is more common than the adults. Oral cavity involvement occurs early in LCH, but the initial symptoms are generally nonspecific, often causing misdiagnosis. This report describes a rare case of chronic localized LCH in an adult patient, with involvement of oral cavity. A 34-year-old male patient presented with multiple nodulo-papular, ulcerated lesions in gingiva involving both the jaws (primarily mandible) and the left buccal mucosa, in addition to regional teeth mobility. The most striking feature was that even after extraction of mobile teeth, the lesions persisted. After recording proper history, performing clinical and radiological evaluation, an incisional biopsy was performed followed by histopathology and immunohistochemistry to reach a confirmatory diagnosis of LCH, thereby implementing early and appropriate initiation of treatment.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的血液系统疾病,其特征是朗格汉斯型组织细胞的局部或全身不受控制的增殖和浸润。它代表了一系列临床病理疾病,从高度侵袭性且常致命的多系统疾病到易于治愈的孤立性骨病变。儿童和较年轻年龄组的受累比成年人更常见。口腔受累在LCH中出现较早,但初始症状通常不具有特异性,常导致误诊。本报告描述了一例成年患者慢性局限性LCH累及口腔的罕见病例。一名34岁男性患者牙龈出现多个结节状丘疹性溃疡病变,累及上下颌(主要是下颌骨)和左侧颊黏膜,伴有局部牙齿松动。最显著的特征是,即使拔除了松动牙齿,病变仍持续存在。在记录了适当病史、进行了临床和放射学评估后,进行了切开活检,随后进行组织病理学和免疫组织化学检查以确诊LCH,从而尽早并适当地开始治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4578/6974994/2b843a6759c8/CCD-10-154-g001.jpg

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