Sun Chuanyin, Xu Guanhua, Lin Jin
Clin Lab. 2018 Oct 1;64(10):1671-1678. doi: 10.7754/Clin.Lab.2018.180421.
It can be difficult to distinguish between IgG4-related lymphadenopathy and multicentric Castleman's disease (MCD) because these conditions cannot be differentially diagnosed using immunohistochemical staining alone. In this study, we analyzed the clinical features of IgG4-related lymphadenopathy and MCD patients.
We retrospectively analyzed 27 patients with MCD, including 20 with plasma cell-type (PC-type) and 7 with hyaline vascular (HV) features (mixed-type). An additional 15 patients with IgG4-related lymphadenopathy were enrolled. Clinical data and immune pathological characteristics, including serum interleukin-6 (IL-6) levels, lymph node lesion biopsies, IgG4+/IgG+ expression, and 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) images, were collected.
The serum levels of C-reactive protein (CRP), IgA, and IL-6 were significantly elevated in the PC/mixedtype group compared with the IgG4-related lymphadenopathy group (p < 0.05). By contrast, the mean age, eosinophilia, globulin, and serum levels of IgG and IgG4 were significantly higher in the IgG4-RD lymphadenopathy group (all p < 0.05). Thirty percent of patients with IgG4-RD lymphadenopathy had elevated IL-6 levels, and 50% with MCD had elevated serum IgG4 levels. Immunohistochemical studies demonstrated the presence of numerous IgG4+ plasma cells, which accounted for > 40% of IgG4/IgG+ cells in 7 of 27 cases in the PC/mixed-type group. We first found that the mean maximum standard uptake value (SUVmax) was strongly associated with albumin and IL-6 in the IgG4-RD lymphadenopathy group, but not in the MCD group. The number of involved organs, but not the standard uptake value (SUV), helped to distinguish between the two diseases. Most PC/mixed-type group patients responded poorly to glucocorticoids when administered alone or in combination with immunosuppressant drugs.
MCD cannot be differentiated from IgG4-related lymphadenopathy using histology alone. Systematic comparative analysis; clinical and laboratory analyses, especially 18F-FDG-PET/CT; and responses to drug treatment are therefore important parameters for distinguishing between these two diseases.
IgG4相关性淋巴结病与多中心Castleman病（MCD）难以区分，因为仅通过免疫组织化学染色无法对这两种疾病进行鉴别诊断。在本研究中，我们分析了IgG4相关性淋巴结病和MCD患者的临床特征。
我们回顾性分析了27例MCD患者，其中20例为浆细胞型（PC型），7例具有透明血管（HV）特征（混合型）。另外纳入了15例IgG4相关性淋巴结病患者。收集临床数据和免疫病理特征，包括血清白细胞介素-6（IL-6）水平、淋巴结病变活检、IgG4+/IgG+表达以及18F-氟脱氧葡萄糖（FDG）正电子发射断层扫描/计算机断层扫描（PET/CT）图像。
与IgG4相关性淋巴结病组相比，PC/混合型组的血清C反应蛋白（CRP）、IgA和IL-6水平显著升高（p < 0.05）。相比之下，IgG4-RD淋巴结病组的平均年龄、嗜酸性粒细胞增多、球蛋白以及IgG和IgG4的血清水平显著更高（均p < 0.05）。30%的IgG4-RD淋巴结病患者IL-6水平升高，50%的MCD患者血清IgG4水平升高。免疫组织化学研究显示，PC/混合型组27例中有7例存在大量IgG4+浆细胞，占IgG4/IgG+细胞的> 40%。我们首次发现，IgG4-RD淋巴结病组的平均最大标准摄取值（SUVmax）与白蛋白和IL-6密切相关，而MCD组则不然。受累器官的数量而非标准摄取值（SUV）有助于区分这两种疾病。大多数PC/混合型组患者单独使用糖皮质激素或与免疫抑制药物联合使用时，对糖皮质激素反应不佳。
仅使用组织学无法将MCD与IgG4相关性淋巴结病区分开来。因此，系统的对比分析、临床和实验室分析，尤其是18F-FDG-PET/CT，以及对药物治疗的反应是区分这两种疾病的重要参数。
