Lymphadenopathy of IgG4-related sclerosing disease
Lymphadenopathy of IgG4-related sclerosing disease
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DOI：
10.1097/pas.0b013e318157c068
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发表时间：
2008-05-01
影响因子：
5.6
通讯作者：
Chan, John K. C.
中科院分区：
文献类型：
--
作者：
Cheuk, Wah;Yuen, Hunter K. L.;Chan, John K. C.
IgG4相关硬化性疾病是最近认识到的一种综合征，其特征是外分泌腺或结外组织由于淋巴浆细胞浸润和硬化而形成肿块，血清IgG4水平升高以及相关组织中IgG4+浆细胞增加。我们报告了该综合征患者局部淋巴结肿大 (n = 3) 和非区域淋巴结肿大 (n = 3) 的形态学特征。患者患有自身免疫性胰腺炎、淋巴浆细胞性硬化性胆管炎、慢性硬化性泪腺炎或慢性硬化性唾液腺炎。淋巴结的组织学特征可分为 3 种模式：Castleman 病样、滤泡增生以及免疫母细胞和浆细胞导致的滤泡间扩张。 IgG4+/IgG+ 浆细胞的百分比显着升高（在包含多种反应条件的 54 个对照淋巴结中，平均为 62%，而 9.9%）。我们还报告了 6 例原发性淋巴结病，其特征是 IgG4+/IgG+ 浆细胞增多（平均 58%）。这些病例与 IgG4 相关的硬化性疾病有许多临床和病理相似之处。事实上，其中 2 名患者在临床过程中出现淋巴浆细胞硬化性胆管炎或泪腺和颌下腺受累。因此，这些病例可能代表了该疾病的原发淋巴结表现。识别 IgG4 相关硬化性疾病的淋巴结病形式的重要性在于对类固醇治疗的显着反应，以及临床或组织学上将该疾病误认为淋巴瘤的可能性。
IgG4-related sclerosing disease is a recently recognized syndrome characterized by mass-forming lesions in exocrine glands or extranodal tissues due to lymphoplasmacytic infiltrates and sclerosis, a raised serum IgG4 level and increased IgG4+ plasma cells in the involved tissues. We report the morphologic features of the enlarged regional (n = 3) and nonregional lymph nodes (n = 3) in patients with this syndrome. The patients presented with autoimmune pancreatitis, lymphoplasmacytic sclerosing cholangitis, chronic sclerosing dacryoadenitis, or chronic sclerosing sialadenitis. The histologic features of the lymph nodes could be categorized into 3 patterns: Castleman diseaselike, follicular hyperplasia, and interfollicular expansion by immunoblasts and plasma cells. The percentage of IgG4+/IgG+ plasma cells was markedly elevated (mean 62% vs. 9.9% in 54 control lymph nodes comprising a wide variety of reactive conditions). We also report 6 cases of, primary lymphadenopathy characterized by increased IgG4+/IgG+ plasma cells (mean 58%). These cases share many clinical and pathologic similarities with IgG4-related sclerosing disease. In fact, 2 of these patients developed lymphoplasmacytic sclerosing cholangitis or lacrimal and submandibular gland involvement during the clinical course. These cases therefore probably represent primary lymph node manifestation of the disease. The importance of recognition of the lymphadenopathic form of IgG4-related sclerosing disease lies in the remarkable response to steroid therapy, and the potential of mistaking the disease for lymphoma either clinically or histologically.