眼眶结外Rosai-Dorfman病伴IgG4阳性浆细胞增多1例

A case of orbital extranodal Rosai-Dorfman disease with increased IgG4 positive plasma cells

患者男性,52岁,右眼肿胀6年余,曾被诊断为"右眼炎性假瘤(泪腺型)"进行手术切除。因右眼睑再次肿胀伴眼球突出加重入院。患者双眼高度近视,右眼泪腺区有肿物,影像学检查示右眼眶泪腺区肿物相关表现。术中见右眼眶肿物特征,术后病理学检查发现病变组织有组织细胞、淋巴细胞、浆细胞增生等情况,免疫组织化学检查有相应结果,基因重排PCR检测结果为未见IgH及IgK克隆性扩增峰,最后诊断为结外Rosai-Dorfman病伴IgG4阳性浆细胞增多,随访3年患者情况良好,肿物无复发。

The patient was a 52-year-old male.His right eye had been swollen for more than 6 years and he had once been diagnosed with"inflammatory pseudotumor of the right eye(lacrimal gland type)"and underwent surgical resection.He was admitted to the hospital due to the recurrence of swelling in the right eyelid accompanied by aggravated exophthalmos.The patient had high-myopia in both eyes,and there was a mass in the lacrimal gland area of his right eye.Imaging examinations showed relevant manifestations of the mass in the lacrimal gland area of the right orbit.During the operation,the characteristics of the mass in the right orbit were observed.Post-operative pathological examinations found that there were hyperplasia of histiocytes,lymphocytes,plasma cells,etc.in the diseased tissues.Immunohistochemical examinations had corresponding results.The result of gene rearrangement PCR detection was that no clonal amplification peaks of IgH and IgK were found.Finally,the patient was diagnosed with extranodal Rosai-Dorfman disease accompanied by an increase in IgG4-positive plasma cells.After 3-year follow-up,the patient was in good condition and there was no recurrence of the mass.