慢性淋巴细胞性白血病(CLL)是成人白血病中最常见的一种。IGVH基因的突变状态是CLL患者最重要的预后标记,而Zeta链相关蛋白激酶(ZAP-70)可作为IGVH突变状态的替代标记。CD38是一种促使B细胞活化和增殖的Ⅱ类跨膜糖蛋白,能提高CLL细胞的生存率,促进其增殖,也可作为CLL的独立预后指标。另外,超过80%的CLL患者存在染色体畸变,最常见的异常是染色体的丢失,其中13q14缺失最为常见(35%);而最常见的染色体增加为12q的三体性(23%)。人类白细胞抗原G(HLA-G)表达增高预示CLL的恶性进展及其生存期的缩短。Toll样受体是天然免疫的重要组成部分,TLR激动剂与放化疗、单克隆抗体,以及肿瘤疫苗联合应用将给CLL的治疗带来重大突破。
Chronic lymphocytic leukemia(CLL) remains the most common adult leukemia.The recent progress on research of molecular and cellular genetics of CLL promotes the development of the diagnosis,treatment and prognosis for CLL patients.IGVH gene mutation status is the most important prognostic marker for CLL patients.Zeta-chain-associated protein kinase(ZAP-70) can be used as a surrogate marker for IGVH mutation status.CD38 is a type Ⅱ transmembrane glycoprotein promoting B cell activation and proliferation,which can improve the survival of CLL cells and enhance their proliferation,so it also can be used as an independent prognostic indicator for CLL.Chromosome aberrations are found in more than 80% of CLL cases.The most frequent abnormalities are losses of chromosomal material,with deletions in band 13q14 being the most common.The most common gains of chromosomal material are trisomies 12q.Human leukocyte antigen G(HLA-G) is a non-classical HLA-I gene.Increased expression of HLA-G leads to the malignant progression of CLL,significantly shortens survival,indicating HLA-G might serve as a prognostic marker in CLL.Toll-like receptors(TLA) are important component of natural immunity.The combination of TLR agonists and release chemotherapy,monoclonal antibodies and tumor vaccines would bring a breakthrough for the treatment of CLL.