摘要
干扰素调节因子4(interferon regulatory factor 4,IRF4)在B细胞发育和分化中具有重要作用,伴IRF4重排的大 B 细胞淋巴瘤(large B-cell lymphoma with IRF4 gene rearrangement,LBCL-IRF4)是近年新发现的一种生发中心来源的成熟B细胞淋巴瘤,最初归入滤泡性淋巴瘤的特殊暂定类型,随着研究和认识的深入,发现其有别于经典型滤泡性淋巴瘤(classic follicular lymphoma,cFL)、儿童型滤泡性淋巴瘤(pediatric follicular lymphoma,PTFL)和弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL).第五版WHO造血与淋巴组织肿瘤分类中正式将此肿瘤归入大B细胞淋巴瘤项下的一个独立类型.为了较全面认识该肿瘤,该文对LBCL-IRF4的临床病理学及分子遗传学特征等研究进展进行总结分析.
Abstract
Interferon regulatory factor 4(IRF4)plays an important role in B-cell development and differentiation.Large B-cell lymphoma with IRF4 gene rearrangement(LBCL-IRF4)is a germinal center derived mature B-cell lym-phoma,initially classified as a special provisional type of follicular lymphoma.With advancing research and under-standing,LBCL-IRF4 has been shown to differ from classic follicular lymphoma(cFL),pediatric follicular lymphoma(PTFL)and diffuse large B-cell lymphoma(DLBCL).The latest fifth edition of the World Health Organization(WHO)classification of hematopoietic and lymphoid tissue tumors has officially classified this tumor as a distinct type under large B cell lymphoma.To gain a more comprehensive understanding of this tumor,this paper reviews the re-search progress on the clinicopathological and molecular genetic characteristics of LBCL-IRF4.