摘要目的：探讨Rosai－Dorfman病病变组织免疫球蛋白G4（IgG4）阳性浆细胞表达情况及其与IgG4相关硬化性疾病（ IgG4－SD）的相关性。方法收集来源于9例Rosai－Dorfman病患者12处病变组织（其中11处淋巴结外，1处淋巴结内）的临床及病理组织学资料，光镜下观察病变组织纤维化程度及闭塞性静脉炎，并采用免疫组织化学EnVision二步法标记其病变组织中IgG4阳性及IgG阳性的浆细胞，用医学图像分析系统定量分析IgG4阳性和IgG阳性的浆细胞含量。结果9处病变组织中有不同程度纤维化（轻度4处，中度1处，重度4处），2处病变组织中见闭塞性静脉炎。12处病变组织中IgG4阳性浆细胞浸润程度如下：重度（＞50／HPF ）4处，中度[（30～50）／HPF ]2处，轻度[（10～29）／HPF]3处，阴性（＜10／HPF）3处，经统计学分析各组间差异有统计学意义（P＜0．01），其中3处病变组织符合IgG4－SD组织学诊断标准，即IgG4阳性浆细胞＞50／HPF、IgG4阳性／IgG阳性浆细胞＞40％，包括左面部皮肤1处、左眼眶外上方1处、右腮腺1处。结论 Rosai－Dorfman病中部分病例符合IgG4－SD IgG4阳性浆细胞的诊断标准，并具有IgG4－SD的病理学组织特征，可能为IgG4－SD谱系中的一员，检测Rosai－Dorfman病组织中IgG4阳性浆细胞可能具有一定的临床病理意义。

abstractsObjective To study the prevalence of IgG 4-positive plasma cells in Rosai-Dorfman disease and to assess the association between Rosai-Dorfman disease and IgG4-related sclerosing disease (IgG4-SD) .Methods The clinicopathologic features of 12 tissue samples of Rosai-Dorfman disease (11 extranodal and one nodal) from nine patients were reviewed.The degree of fibrosis and occlusive phlebitis was studied by HE staining .The expression of IgG4 and IgG in plasma cells were studied by immunohistochemistry ( EnVision ) and quantitatively analyzed by medical image analysis system .Results Nine tissue samples showed different degree of fibrosis ( four tissue samples were mild , one tissue sample was moderate and four tissue samples were severe ) and two tissue samples showed occlusive phlebitis in the lesional tissue.Immunohistochemical study showed marked infiltration by IgG 4-positive plasma cells (>50 per high-power field ) in four tissue samples , moderate infiltration ( 30 to 50 per high-power field ) in two tissue samples,mild (10 to 29 per high-power field) in three cases and negative infiltration (<10 per high-power field) in three tissue samples (P <0.01).Three tissue samples fulfilled the diagnostic criteria of IgG4-SD (>50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio >40%), including one tissue sample each of Rosai-Dorfman disease in the left facial skin , above the left eye socket , and in the right parotid.Conclusions Some cases of Rosai-Dorfman disease fulfill the diagnostic criteria and show the histologic features of IgG 4-SD.They may represent members of the IgG 4-SD spectrum.The detection of IgG4-positive plasma cells in the lesional tissues of Rosai-Dorfman disease may have clinical pathological significance.