成年人Still病三例淋巴结、骨髓和肝脏病理学特征分析

Pathologic changes of lymph nodes, liver and bone marrow in adult-onset Still＇s disease： a report of 3 cases

目的 探讨成年人Still病（AOSD）的淋巴结、骨髓和肝脏病理学特征.方法 收集3例AOSD患者的临床病理资料,对其活组织检查的淋巴结和骨髓、肝脏进行组织形态学观察及免疫组织化学分析.结果 AOSD肿大淋巴结呈高度增生改变,淋巴结部分结构破坏,副皮质区淋巴结组织增生明显,伴有免疫母细胞和活化的小淋巴细胞、朗格汉斯细胞和组织细胞弥散增生、大量中性粒细胞及慢性炎细胞浸润.骨髓活组织检查示粒红比例显著增大,粒系明显核右移.肝穿刺显示肝细胞点灶状坏死,汇管区伴少量淋巴细胞和中性粒细胞浸润.免疫组织化学染色：CD21显示残存的淋巴滤泡,副皮质区免疫母细胞大多数表达B细胞标记,少数表达T细胞标记.结论 对AOSD患者淋巴结、肝脏及骨髓穿刺活组织检查标本的形态学观察有助于其诊断和鉴别诊断.

Objective To investigate the pathological characteristics of lymph nodes,liver and bone morrow in adult-onset still＇s disease （AOSD）.Methods The clinical data of three AOSD patients were collected and their biopsy of enlarged lymph nodes,liver and bone marrow were analyzed by histology and immunohistochemistry.Results In AOSD lymphadenopathy,there were high lymph node hyperplasia,partial architecture damage and an intense paracortical hyperplasia along with diffused infiltration of large immunoblasts,activated lymphocytes,Langerhans cells,histocytes,neutrophils and chronic inflammatory cells.Bone marrow biopsy showed a significant increase in the proportion of granulocytes and erythrocytes,with apparently increased segmented neutrophils.Hepatic biopsy showed hepatocellular spotty necrosis.Meanwhile,there was some infiltration of lymphocytes and neutrophils in the periportal fibrosis area.Immunohistochemical staining showed CD21 expression in lymphoid follicle remnants and B cell markers with few T markers in most of immunoblasts.Conclusion The pathological characterizations of lymph nodes,liver and bone marrow provide important clues to the diagnosis of AOSD as well as its differential diagnosis.