血管内大B细胞淋巴瘤的临床表现和治疗学研究进展

根据最近的WHO分类血管内大B细胞淋巴瘤（IVLBCL）是非霍奇金淋巴瘤罕见的疾病实体。其特征为在各种器官的小血管腔内肿瘤细胞选择性生长。IVLBCL特征包括无明显的淋巴结肿大,通常有侵袭性的临床习性,导致正确诊断延迟和致死结局,预后极差。抗CD-20单抗利妥昔象征着B细胞淋巴瘤治疗学临床实践中的重要里程碑。利妥昔加常规化疗已显示有益,对IVLBCL临床和病理学的理解正在增加。本文阐述当今IVLBCL的探索要点,讨论该病的生物学和临床观点中未解决的问题。

Presentation and management of intravascular large B -cell lymphoma

Intravascular large B -cell lymphoma（IVLBCL） is a rare disease entity of non -Hodgkin lymphoma according to the current WHO classification. This rare form of B - cell lymphoma is characterized by selective growth of turnout cells in the lumina of small vessels of various organs. Strange characteristics of IVLBCL,including the absence of marked lymphoadenopathy and the usually aggressive clinical behaviour, result in the delay of timely and accurate diagnosis and fatal complications. Thus, the prognosis of IVLBCL is extremely poor. The success achieved with the anti -CD20 chimeric monoclonal antibody,rituximab,represets an important milestone in the clinical practice of B -cell Lymphoma. An advantage of adding rituximab to conventional chemotherapies has been shown, in the process of increasing understanding of the clinical and pathological manifestations for IVLBCL. This review describes the cutting edge of research on IVLBCL, and discusses the unsolved issues from biological and clinical perspectives to provide a better understanding of this rare lymphoma.