摘要
ALK阴性的间变性大细胞淋巴瘤(ALK-ALCL)虽然具有和ALK+ALCL相似的形态学特征,CD30亦呈强阳性,但缺乏相对特异的ALK蛋白表达。近来研究表明,两种ALCL不仅在分子和遗传学水平上存在实质性区别,而且在治疗反应,预后和长期生存等方面,ALK-ALCL远远差于ALK+ALCL,如ALK-ALCL的受累人群一般为老年人,大部分患者合并B组症状,诊断时已处于疾病晚期,国际预后指数评分较高,常规治疗疗效较差,5年生存率低于49%等。鉴于此,本文对ALK-ALCL的基础细胞形态和组织病理、免疫麦型、细胞遗传学和分子标靶以及诊疗新进展作一综述。
ALK negative anaplastic large cell lymphoma（ALK-ALCL） lacks the specific expression of ALK protein,although it also strongly expresses CD30 and resembles the morphologic characteristics of ALK positive anaplastic large cell lymphoma（ALK＋ ALCL）.Recently,some new researches indicate that there exist molecular and genetic differences between these two types of ALCL.Moreover,the treatment response,prognosis,and long-term survival of ALK-ALCL are far worse than that of ALK＋ ALCL,such as ALK-ALCL is associated with older age persons,B group syndrome,disease advanced stage,high International Prognostic Index（IPI） and poor prognosis（〈49% 5-year survival）.As a consequence,some new advances on basis（cell morphology and tissue pathology,immunophenotypes,cell genetics and molecular marker）,diagnosis and treatment of ALK-ALCL are summrized in this review.
出处
《中国实验血液学杂志》
CAS
CSCD
2011年第2期511-516,共6页
Journal of Experimental Hematology
基金
江苏省“科教兴卫”工程医学重点人才资助项目(编号RC2007072)
江苏省人事厅”六大人才高峰”资助项目(编号07-B-019)
苏州市国际科技合作项目(编号SWH0815)
苏州市科技计划应用基础项目(编号YJS0912)
