Shrirao Neha, Sethi Aditya, Mukherjee Bipasha
Department of Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Medical Research Foundation, Chennai, Tamil Nadu, India.
J Pediatr Hematol Oncol. 2016 Oct;38(7):e248-50. doi: 10.1097/MPH.0000000000000526.
Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is an extremely rare non-Langerhans cell histiocytosis. Orbital involvement is even rarer and may be accompanied by lymph node involvement. Treatment options range from systemic steroids and immunosuppressants to radiation and debulking. We present a rare case of bilateral orbital sinus histiocytosis with massive lymphadenopathy with cervical and circumaortic lymphadenopathy.
罗萨伊-多夫曼病或伴巨大淋巴结病的窦性组织细胞增多症是一种极其罕见的非朗格汉斯细胞组织细胞增多症。眼眶受累更为罕见,可能伴有淋巴结受累。治疗选择范围从全身用类固醇和免疫抑制剂到放疗和减瘤手术。我们报告一例罕见的双侧眼眶窦性组织细胞增多症伴巨大淋巴结病,同时伴有颈部和主动脉周围淋巴结病。