窦组织细胞增生伴巨大淋巴结病1例并文献复习

Sinus histiocytosis with massive lymphade-nop-athy,SHML 1 case report and literature review

窦组织细胞增生症伴巨大淋巴结病是一种病因不明的良性组织细胞增生性疾病,又称Rosai-Dorfman病。可累及多个器官,临床表现为发热、颈部淋巴结肿大、皮肤黄瘤样斑、结节,伴有白细胞增多、高球蛋白血症、血沉增快。组织病理可见真皮大量组织细胞浸润,在组织细胞的胞浆内可见被吞噬的完整的淋巴细胞、嗜中性粒细胞等。患者皮疹可自行消退,治疗可采用手术切除、口服糖皮质激素、反应停、维甲酸等。

Sinus Histiocytosis with massive lymphadenopathy （SHML） or rosai-dorfman disease is a benign, histiocytic proliferative disorder with undetermined etiology. It usually appears as a febrile illness associated with massive cervical lymphadenopathy, skin lesions, hyperglobulinem ia, leukocytosis and an elevated sedimentation rate. 10% of the patients with SHML have skin lesions, patients with only skin lesions were called ＂cutaneous ro saidorfman disease ＂which manifestated as yellowbrown papules, nodules, and plaques. A very diagnostic feature in histopathology is the finding of intact lymphocyte （or plasma cells） in the cytoplasm of the histiocytie cells which was called ＂emperipolesis＂. The lesionsmay clear spontaneously in some patients, treatment of RDD include surgical excition, systemic corticosteroids, thalidomied and retinoids.