血管内大B细胞淋巴瘤4例临床病理分析

Intravascular Large B-cell Lymphoma:Clinical and Histopathological Studies of 4 Cases

目的探讨血管内大B细胞淋巴瘤的临床和病理特点,以提高其诊治水平。方法收集并分析了4例血管内大B细胞淋巴瘤的临床特征和病理资料,用形态学方法和免疫组化染色进行研究。结果发生于皮肤2例,出现皮肤斑块和破溃;发生于脑部2例,出现神经系统症状。镜下所见相似,瘤细胞仅位于小血管腔内,瘤细胞大,核空泡状,核仁明显,核分裂易见。瘤细胞表达B细胞标记LCA(+),CD20(+),CD79a(+),CD30(-),CD3(-),CD45RO(-),Ⅷ因子,Desmin阴性。结论该瘤是一种罕见的结外弥漫性大B细胞淋巴瘤,诊断只能依靠病理检查确诊。该瘤高度恶性和高度侵袭性,对化疗不敏感,多数患者发病后短期死亡,预后很差,应早期诊断,积极联合化疗。

Objective To investigate the clinical and pathological characteristics of the intravascular large B-cell lymphoma（IVLBCL）and to improve the diagnosis and treatment level of the IVLBCL.Methods Four patients with detailed clinical data, HE staining and immunohistological staining were analyzed.Results Two cases presented as cutaneous symptoms, the other two cases presented as central nervous symptoms.Under microscope, the lumens of the small vessels were filled with neoplastic cells.The tumor cells were similar to the diffuse large B-cell lymphoma（DLBCL）,which were positive for LCA,CD20,CD79a and negative for CK,CD45RO,CD3,CD30,KP-1, Desmin and F Ⅷ Ag.Conclusions IVLBCL is a rare variant of DLBCL,and the diagnosis is based on the histopathology and immunohistochemistry.This extremely malignant and highly aggressive tumor is not sensitive to chemotherapy, with poor prognosis.Early diagnosis and combination chemotherapy may induce a better prognosis.