Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease
Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease
复制标题
DOI：
10.1038/modpathol.2009.17
复制
发表时间：
2009-04-01
期刊：
影响因子：
7.5
通讯作者：
Yoshino, Tadashi
中科院分区：
文献类型：
--
作者：
Sato, Yasuharu;Kojima, Masaru;Yoshino, Tadashi
igg4相关疾病有时累及局部和/或全身性淋巴结，在临床和/或组织学上常与多中心Castleman病或恶性淋巴瘤相似。在这项研究中，我们检查了9例全身igg4相关淋巴结病的临床和病理表现。这些病例均与人类疱疹病毒-8或人类免疫缺陷病毒感染无关，也没有t细胞受体或免疫球蛋白基因重排。组织学上，根据igg4阳性细胞浸润方式将全身性igg4相关淋巴结病分为滤泡间浆细胞增多型和生发中心浆细胞增多型两种。滤泡间浆细胞增多症表现为Castleman病样特征或非典型淋巴浆细胞增生和免疫母细胞增生样特征。生发中心浆细胞增多型表现为明显的滤泡增生，igg4阳性细胞主要向生发中心浸润，部分病例表现为生发中心进行性转化。有趣的是，我们的9例病例中有8例（89%）在受影响的淋巴结中显示嗜酸性粒细胞浸润，并且检查的患者显示血清IgE高升高。实验室检查显示血清IgG4和可溶性白介素-2受体升高。然而，几乎所有患者的白细胞介素-6、c反应蛋白和乳酸脱氢酶水平均在正常范围内或仅轻微升高。1例患者白细胞介素-6水平高，而c反应蛋白在正常范围内。5例患者检测自身抗体，4例患者检测到自身抗体。与先前报道的多中心Castleman病病例相比，我们的全身性igg4相关淋巴结病变患者明显年龄较大，c反应蛋白和白细胞介素-6水平明显降低。总之，在我们的全身性igg4相关淋巴结病中，病理特征仅部分与多中心性Castleman病重叠，血清数据（尤其是c反应蛋白和白细胞介素-6）有助于两者的鉴别。我们的研究结果表明，受影响组织中的嗜酸性粒细胞浸润和血清IgE升高可能提示全身igg4相关淋巴结病的发病机制存在过敏机制。
IgG4-related disease sometimes involves regional and/or systemic lymph nodes, and often clinically and/or histologically mimics multicentric Castleman's disease or malignant lymphoma. In this study, we examined clinical and pathologic findings of nine patients with systemic IgG4-related lymphadenopathy. None of these cases were associated with human herpes virus-8 or human immunodeficiency virus infection, and there was no T-cell receptor or immunoglobulin gene rearrangement. Histologically, systemic IgG4-related lymphadenopathy was classified into two types by the infiltration pattern of IgG4-positive cells: interfollicular plasmacytosis type and intra-germinal center plasmacytosis type. The interfollicular plasmacytosis type showed either Castleman's disease-like features or atypical lymphoplasmacytic and immunoblastic proliferation-like features. By contrast, the intra-germinal center plasmacytosis type showed marked follicular hyperplasia, and infiltration of IgG4-positive cells mainly into the germinal centers, and some cases exhibited features of progressively transformed germinal centers. Interestingly, eight of our nine (89%) cases showed eosinophil infiltration in the affected lymph nodes, and examined patients showed high elevation of serum IgE. Laboratory examinations revealed elevation of serum IgG4 and soluble interleukin-2 receptors. However, the levels of interleukin-6, C-reactive protein, and lactate dehydrogenase were within normal limits or only slightly elevated in almost all patients. One patient showed a high interleukin-6 level whereas C-reactive protein was within the normal limit. Autoantibodies were examined in five patients and detected in four. Compared with the previously reported cases of multicentric Castleman's disease, our patients with systemic IgG4-related lymphadenopathy were significantly older and had significantly lower C-reactive protein and interleukin-6 levels. In conclusion, in our systemic IgG4-related lymphadenopathy showed pathologic features only partially overlapping those of multicentric Castleman's disease, and serum data (especially C-reactive protein and interleukin-6) are useful for differentiating the two. Our findings of eosinophil infiltration in the affected tissue and elevation of serum IgE may suggest an allergic mechanism in the pathogenesis of systemic IgG4-related lymphadenopathy.