【摘 要】
：
Rosai-Dorfman病,又称窦组织细胞增生伴巨大淋巴结病,是一种罕见的组织细胞病,通常表现为青少年的无痛性双侧颈部淋巴结肿大.Rosai-Dorfman病发病累及骨骼者不到10％,并且多达7
【机 构】
：
上海交通大学附属第六人民医院骨科,上海,200233
论文部分内容阅读
Rosai-Dorfman病,又称窦组织细胞增生伴巨大淋巴结病,是一种罕见的组织细胞病,通常表现为青少年的无痛性双侧颈部淋巴结肿大.Rosai-Dorfman病发病累及骨骼者不到10％,并且多达75％的骨骼Rosai-Dorfman病患者同时存在软组织病灶.颅骨、颌面骨和胫骨是骨骼Rosai-Dorfman病最常见的发病部位.该病临床表现缺乏特异性,诊断主要依靠常规病理学和免疫组织化学染色检查.临床表现主要是局部疼痛和肿胀.影像学上,通常表现为髓内的溶解性病变,有时伴有周围硬化.目前,Rosai-Dorfman病的病因尚不明确,可能涉及潜在的宿主免疫失调、IgG4相关疾病、多种自身免疫性疾病和基因突变等.目前伴有症状的骨骼Rosai-Dorfman病的治疗方案主要取决于具体病灶位置,主要包括手术刮除或切除,其他治疗方案包括激素治疗和化疗等.由于骨骼Rosai-Dorfman病的临床和影像学表现通常提示恶性病变可能,部分患者可能接受比较激进的治疗.全身PET/CT可以用于Rosai-Dorfman病的分期、随访和评估.
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