Karakuş Volkan, Dere Yelda Morgül, Soysal Dilek Ersil
Mugla Sıtkı Kocman University Training and Research Hospital, Department of Hematology, Mentese, Mugla 48100, Turkey.
Mugla Sıtkı Kocman University Training and Research Hospital, Department of Pathology, Mentese, Mugla 48100, Turkey.
Case Rep Med. 2020 Feb 27;2020:5727569. doi: 10.1155/2020/5727569. eCollection 2020.
Rosai-Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the enlarged sinusoids of the lymph nodes. The clinical course is unpredictable, but is often benign with spontaneous resolution of disease in most patients. We report a patient with bilateral massive enlargement of cervical, axillary, and inguinal lymph nodes, moderately enlarged spleen, and a weight loss of 15 kg. Excisional biopsy from the cervical lymph node showed that the dilated sinusoids were infiltrated by lymphocytes, plasma cells, and large histiocytes with CD 68 and S-100 protein positive. Due to the slow progression of the disease, oral prednisolone with a body weight of 1 mg/kg was started in March 2016. The steroid dosage has been adjusted many times during the clinical follow-up. After 33 months, steroid treatment resulted in partial shrinkage of lymph nodes, the spleen returned to its normal size, and the patient gained weight. After 38 months of follow-up, no systemic symptoms, sign, or extranodal involvement were detected, and the patient continued with low-dose steroid treatment.
罗萨伊-多夫曼病（RDD）是一种病因不明的罕见良性鼻窦组织细胞增生症。淋巴结病是主要临床表现，但其他多种器官也可受累。组织学特征包括在淋巴结扩大的窦内出现具有特征性核特征的S-100+组织细胞。临床病程不可预测，但多数患者病情通常为良性且可自发缓解。我们报告一例患者，其颈部、腋窝和腹股沟淋巴结双侧大量肿大，脾脏中度肿大，体重减轻15千克。颈部淋巴结切除活检显示，扩张的窦内有淋巴细胞、浆细胞和大组织细胞浸润，CD 68和S-100蛋白呈阳性。由于疾病进展缓慢，2016年3月开始口服泼尼松龙，剂量为1毫克/千克体重。在临床随访期间，类固醇剂量多次调整。33个月后，类固醇治疗使淋巴结部分缩小，脾脏恢复正常大小，患者体重增加。随访38个月后，未发现全身症状、体征或结外受累情况，患者继续接受小剂量类固醇治疗。
