Abstract

目的: 观察并总结脾硬化性血管瘤样结节性转化的病理形态,探讨其与IgG4相关性疾病的关系。 方法: 收集北京医院诊断的脾硬化性血管瘤样结节性转化共3例,总结临床病历资料、病理特点,并复习相关文献。 结果: 本研究病例均为女性,年龄38~63岁,患者均无临床症状,CT显示肿物呈低密度影,其中1例患者术前血清IgG及IgG4水平均显著升高。脾脏大体检查可见肿物呈结节状,切面呈灰白色间灰红色或灰褐色,肿物最大径4.2~5.0 cm,境界较清楚。低倍镜下肿物呈多结节状,结节内可以看到窦隙样血管腔隙和肥胖的内皮细胞,可见3种不同类型的血管。结节周围染色较淡,纤维组织呈向心性增生,并伴有淋巴细胞、浆细胞浸润。2例肿物中IgG4阳性的浆细胞数量均>10个/HPF。 结论: 脾硬化性血管瘤样结节性转化是罕见的良性肿瘤样病变,发病比较隐匿,诊断依赖病理诊断,发病机制不明,其与IgG4相关性疾病的关系尚需进一步研究。.

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