眼附属器淋巴造血组织增生性疾病临床病理分析

Analysis on clinicopathologic characteristics of lymphoid and hemopoietic tissue proliferation disease in orbital and ocular adnexal

目的探讨眼附属器淋巴造血组织增生性疾病的临床病理学特征。方法回顾性分析210例首都医科大学附属北京同仁医院眼附属器淋巴造血组织增生性疾病患者的临床资料和临床病理资料。结果 1该组眼附属器淋巴造血组织增生性疾病中最常见的是非霍奇金淋巴瘤(100例,47.6%),其次为炎性假瘤(45例,21.4%)和良性淋巴上皮病变(Mikuliczs病,41例,19.5%)。2该组病变部分类型在发病年龄和性别上有特点,组织病理学上的表现有交叉。3该部位非霍奇金淋巴瘤中最常见的为结外黏膜相关组织边缘区B细胞淋巴瘤(74例),眼附属器的结外黏膜相关组织边缘区B细胞淋巴瘤有其不同于其他解剖部位的该肿瘤的特点。4免疫组织化学染色在该部位淋巴造血组织增生性疾病的诊断和鉴别诊断中有非常重要的作用。5眼附属器的Ig G4相关性疾病(10例)的诊断和临床病理表现有其独有的特征。结论眼附属器淋巴造血组织增生性疾病是一类比较常见的疾病,其在发病年龄、性别等临床表现和组织学类型上都具有明显的临床病理学特征,掌握这些对临床病理诊断将提供较大的帮助。

Objective To study the clinicopathologic characteristics of lymphoid and hemopoietic tissue proliferation disease in orbital and ocular adnexal. Methods Clinical manifestations and the characteristics of clinicopathology were analyzed retrospectively in two hundreds and ten patients suffering from lymphoid and hemopoietic tissue proliferation in orbital and ocular adnexal. Results The most common histologic type in orbital and ocular adnexa is primary extraocular non-Hodgkin＇s lymphoma, secondly was Mikuliczs disease and inflammatory pseudotumor. There is a characteristic in age and gender among the various types of this group, and there is an overlap in clinicopathologic characteristics. The most common histologic subtype was extraocular marginal zone lymphoma of mucosa-associated lymphoid tissue type in extraocular non-Hodgkin＇ s lymphoma, which accounted for 74. 0% （74 / 100 cases） of cases. The extraocular marginal zone lymphoma of mucosa-associated lymphoid tissue type has different traits from that at other sites. Immunohistochemical staining is very important to the diagnosis and differential diagnosis for lymphoid and hemopoietic tissue proliferation disease in this site. The unique feature in diagnosis and clinicopathology is obvious to IgG4-related disease of this site. Conclusion We conclude that lymphoid and hemopoietic tissue proliferation disease in orbital and ocular adnexal is common among patients. There is a characteristic in age, gender and histologic subtype, which is helpful to understand these characteristics for pathological diagnosis.