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一例罕见的口腔多系统朗格汉斯细胞组织细胞增多症。

A rare case of oral multisystem Langerhans cell histiocytosis.

作者信息

Facciolo Maria-Teresa, Riva Francesco, Gallenzi Patrizia, Patini Romeo, Gaglioti Domenico

机构信息

Oral Surgery Unit of George Eastman Hospital, Umberto I Teaching Hospital, Rome, Italy.

Clinical Dentistry Institute of Head and Neck Clinical Area. School of dentistry, Catholic University of Sacred Heart. Largo A. Gemelli, 1 - 00168 Rome, Italy.

出版信息

J Clin Exp Dent. 2017 Jun 1;9(6):e820-e824. doi: 10.4317/jced.53774. eCollection 2017 Jun.

Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by high proliferation of Langerhans dendritic cells. LCH is a solitary or multifocal disease that primarily involves bone tissue and often affects children and young men. A 29 years-old Caucasian man was referred to the Oral Surgery Unit of George Eastman Hospital - Umberto I teaching hospital, with third degree mobility of teeth belonging to second, third and fourth quadrant. Panoramic radiograph showed multiple radiolucent areas with well demarcated borders on the right and left site of the mandible and on the left site of the maxilla. Extractions of compromised teeth and biopsy of the osteolytic tissue were performed. The final diagnosis of multisystem Langerhans cell histiocytosis of the soft and hard tissues of the oral cavity was made. The patient was sent to the Hematology department of Umberto I Teaching Hospital of "Sapienza" - University of Rome for the proper treatment. The present case of rare multisystem LCH involving oral hard and soft tissues shows the strong importance of better investigate, with appropriate additional exams, initial shifty symptoms that could lead to a misdiagnosis. Differential diagnosis, microscopic diagnosis, Langerhans cell histiocytosis.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的疾病,其特征为朗格汉斯树突状细胞高度增殖。LCH是一种单发性或多灶性疾病,主要累及骨组织,且常影响儿童和青年男性。一名29岁的白种男性被转诊至乔治·伊斯曼医院 - 翁贝托一世教学医院的口腔外科,其第二、第三和第四象限的牙齿出现三度松动。全景X线片显示下颌骨左右两侧及上颌骨左侧有多个边界清晰的透射区。对患牙进行了拔除,并对溶骨性组织进行了活检。最终诊断为口腔软硬组织多系统朗格汉斯细胞组织细胞增多症。该患者被送往罗马“萨皮恩扎”大学翁贝托一世教学医院的血液科进行适当治疗。本例罕见的累及口腔软硬组织的多系统LCH病例表明,对于可能导致误诊的初始可疑症状,通过适当的额外检查进行更好的排查非常重要。鉴别诊断、显微镜诊断、朗格汉斯细胞组织细胞增多症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9cc8/5474341/36fc3938dcbd/jced-9-e820-g001.jpg

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