Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, People's Republic of China.
Hematology. 2021 Dec;26(1):552-555. doi: 10.1080/16078454.2021.1941552.
Rosai-Dorfman disease (RDD) is a benign and rare non-Langerhans cell histiocytic proliferative disorder. Laryngeal involvement is an unusual site of extranodal involvement of RDD. Laryngeal RDD can cause life-threatening airway obstruction that requires effective control of the disease. In this study, we report three cases of laryngeal RDD with excellent and durable responses to thalidomide.
Patient 1 was a 39-year-old male who presented with a two-year history of nasal obstruction. Patient 2 was a 26-year-old woman who presented complaining of a hoarse voice for one year. Patient 3 was a 24-year-old man who presented with complaints of a hoarse voice and progressing dyspnea for five months. Electronic laryngoscopy revealed submucous nodular lesions in the nasal cavity, nasopharynx, and larynx of the three patients. Biopsy of the lesions showed large histiocytes with abundant pale cytoplasm which were S-100 and CD68 positive consistent with RDD.
Before thalidomide treatment, patient 1 received chemotherapy and six times surgical excision due to the recurrence of laryngeal lesions. Patient 2 failed steroid treatment. Patient 3 underwent an emergency tracheostomy due to airway obstruction. All three patients then received thalidomide 100 mg/d treatment and achieved satisfactory and durable responses with the longest follow-up of 45 months.
Thalidomide may induce long-term remission in laryngeal RDD.
罗萨-多夫曼病（RDD）是一种良性且罕见的非朗格汉斯细胞组织细胞增生性疾病。喉部受累是 RDD 结外累及的不常见部位。喉部 RDD 可导致危及生命的气道阻塞，需要有效控制疾病。本研究报告了三例喉 RDD 患者，他们对沙利度胺有极好且持久的反应。
患者 1 为 39 岁男性，有两年的鼻塞史。患者 2 为 26 岁女性，因声嘶一年就诊。患者 3 为 24 岁男性，因声嘶和进行性呼吸困难 5 个月就诊。电子喉镜显示三名患者的鼻腔、鼻咽和喉部有粘膜下结节状病变。病变活检显示大组织细胞，胞浆丰富苍白，S-100 和 CD68 阳性，符合 RDD。
在接受沙利度胺治疗之前，患者 1 因喉部病变复发接受了化疗和六次手术切除。患者 2 对类固醇治疗无效。患者 3 因气道阻塞而行紧急气管切开术。所有三名患者随后接受沙利度胺 100mg/d 治疗，并获得了满意且持久的反应，最长随访时间为 45 个月。
沙利度胺可能诱导喉部 RDD 长期缓解。
