Rosai Dorfman Disease in Mandible: A Rare Case Report.

Hassan Mirmohammad Sadeghi, Roozbeh Azadi, Mehrdad Dehghanpour Barouj
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Abstract

Rosai Dorfman disease is generally defined as a massive bilateral painless cervical lymphadenopathy accompanied with both fever and leukocytosis with neutrophilia. Additionally, it may possibly be associated with polyclonal hypergammaglobulinemia, reversal of CD4/CD8 ratio, the elevated erythrocyte sedimentation rate (ESR), microcytic anemia, and thrombocytosis. Rosai-Dorfman disease is known as a benign self-limiting disease, so no treatment is required in many cases, although it causes death in some cases by involving vital organs like kidney. The treatment is required when there is a life-threatening situation such as airway obstruction or involvement of vital organs such as kidney, liver, and lower respiratory tract. The required treatment choices include steroid therapy, chemotherapy, radiotherapy, and surgery. Surgical treatment is performed for bulk removal to resolve the obstruction caused by the mass as well as taking biopsy for the definite histopathologic diagnosis of disease. A 26-year-old man was referred to oral and maxillofacial surgery (OMFS) clinic of Taleghani hospital with chief complaints of pain and swelling of left submandibular space. According to the patient himself, the swelling had been started three months earlier. After rejecting dental source of the lesion, we decided to remove the mass by excisional biopsy concerning the patient's discomfort. Histopathology report verified Rosai Dorfman disease as definite diagnosis of the mass.

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下颌骨Rosai Dorfman病1例报告。
Rosai Dorfman病通常定义为双侧无痛性颈淋巴肿大伴发热、白细胞增多伴中性粒细胞增多。此外,它可能与多克隆高γ球蛋白血症、CD4/CD8比值逆转、红细胞沉降率(ESR)升高、小细胞性贫血和血小板增多有关。罗赛-多尔夫曼病被认为是一种良性的自限性疾病,因此在许多情况下不需要治疗,尽管在某些情况下,它会因累及肾脏等重要器官而导致死亡。当出现危及生命的情况时,如气道阻塞或重要器官(如肾、肝和下呼吸道)受累时,需要进行治疗。所需的治疗选择包括类固醇治疗、化疗、放疗和手术。手术切除肿块以消除肿块引起的梗阻,同时进行活检以明确疾病的组织病理学诊断。一名26岁男子被转介到Taleghani医院口腔颌面外科(OMFS)诊所,主诉为左侧下颌下间隙疼痛和肿胀。据病人自己说,肿胀是三个月前开始的。考虑到患者的不适,在拒绝了病变的牙源后,我们决定通过切除活检切除肿块。组织病理学报告证实Rosai Dorfman病是肿块的明确诊断。
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