成人Still病的淋巴结病理诊断及临床意义

目的 观察成人Still病（AOSD）的淋巴结病理形态特点,探讨其在AOSD诊断中的临床意义.方法 对浙江省桐乡市中医医院病理科收治的4例AOSD患者的临床资料进行回顾性分析,并对其淋巴结活检及免疫组化标记结果进行观察分析.结果 患者主要表现为体温升高、皮疹及关节痛等临床症状,肝脾及淋巴结肿大,外周血白细胞较正常水平升高,EB病毒,HIV及抗RNP抗体正常.患者淋巴结部分结构保存,副皮质区增宽,淋巴结主要呈增生性改变,增生细胞主要是免疫母细胞及Langerhans细胞,可见小淋巴细胞及组织细胞等散在分布,增生细胞核分裂象较多见,且异型性明显.免疫组化标记结果显示,免疫母细胞表达CD20及CD79α或表达CD3及CD45RO,Langerhans细胞胞质及胞核、高尔基区,CD1a和vementin胞质阳性,活化的淋巴细胞表达CD30,组织细胞表达CD68,CD15及ALK等均无表达.结论 AOSD临床诊断不依赖于淋巴结活检,但淋巴结活检对AOSD的诊断具有重要的辅助价值,且肿大的淋巴结的免疫反应特点对AOSD的鉴别诊断也具有重要意义.

Lymph node pathology diagnosis and clinical significance of adult-onset Still's desease

Objective To observe the pathological features of lymph nodes from adult-onset Still＇s desease （AOSD）,and explore its clinical significance in the diagnosis of AOSD.Methods A retrospective analysis of 4 cases with AOSD treated in Department of Pathology,Tongxiang TCM Hospital of Zhejiang Province was made,and the results of its lymph node biopsy and immunohistochemical markers were observed and analyzed.Results The main symptoms of patients were fervescence,skin rash,joint pain and other clinical symptoms,and liver,spleen and lymph node enlargement.The count of peripheral blood leucocyte was higher than normal levels.The EB virus,HIV,and anti-RNP antibodies were well behaved.Some structure of lymph nodes was saved,and the paracortex of it became broadened.The major changes were hyperplastic lymph nodes,and the proliferating cells mainly contain immunoblasts and Langerhans cells,and some small lymphocytes and tissue cells were diffusely distributed among them.The mitotic figures of proliferating cell nuclear were more common,and marked pleomorphism.The result of immunohistochemical markers showed that the immunoblasts could express CD20 and CD79α or CD3 and CD45RO,the cytoplasm,nucleus and golgiosome,CDla and vementin of Langerhans cells reacted positive.The activated lymphocyte expressed CD30 and the histocyte expressed CD68.Otherwise,the CD15 and ALK were not expressed.Conclusion The clinical diagnosis of AOSD is not dependent on the lymph node biopsies,but the lymph node biopsies has an important auxiliary value on the diagnosis of AOSD.And the immune response characteristics of the enlarged lymph nodes is also important for the differential diagnosis of AOSD.