成人Still病的淋巴结病理诊断及临床意义

Pathologic changes of lymph nodes in adult-onset Still's disease:diagnosis and clinical significance

目的探讨成人Still病(adult onset Still’s disease,AOSD)淋巴结的病理形态学特点及临床意义。方法收集3例AOSD患者的临床资料,对其活检的淋巴结行组织学观察及免疫组织化学分析。结果 (1)临床特点:高热、皮疹和关节痛,伴肝、脾及淋巴结肿大;辅助检查:血WBC≥15×109/L,N≥0.8,SF>1 200 ng/ml。(2)AOSD肿大淋巴结呈高度增生改变。淋巴结结构部分保存,副皮质区免疫母细胞和(或)Langerhans细胞弥漫增生,同时伴有组织细胞、小淋巴细胞及炎细胞的混杂性增生,增生细胞异型性明显,核分裂象多见;免疫组化染色:增生的免疫母细胞T、B淋巴细胞标记混合阳性,Langerhans细胞S-100蛋白、CD1α和vimentin阳性,组织细胞表达CD68,活化的淋巴细胞CD30阳性。结论临床表现与辅助检查对AOSD的诊断至关重要。肿大淋巴结过度的免疫反应表现对诊断也有重要提示意义和鉴别诊断价值。

Purpose To investigate the pathological characteristics and diagnosis of adult-onset Still＇s disease（AOSD）.Methods Three cases of the lymph nodes of AOSD were studied with histology,immunohistochemistry,and analyses of their clinical features.Results AOSD was clinically characterized by fever,skin rash,arthralgias,leukocytosis and the serum level of ferritin exceeded 1200 ng/ml.The enlarged lymph nodes were highly hyperplastic.The normal architectures of lymph nodes were partially kept and characterized by prominent paracortical hyperplasia,including diffuse infiltration of large immunoblasts,Langerhans cells,histiocytes,reactive lymphocytes and inflammatory cells.Some of the proliferative cells were markedly pleomorphic and atypical,and mitotic figures were usually common.The immunoblasts showed mixed expression for T and B markers by immunohistochemistry.Langerhans cells were immunoreactive for S-100,CD1α and vimentin.Histiocytes were positive for CD68 and the activated lymphocytes for CD30.Conclusions Clinical features and auxiliary examination are vital to the diagnosis of AOSD.The excessive immunoreactions of the enlarged lymph nodes provide important clues to the diagnosis of AOSD and differential diagnosis as well.