美国国立卫生研究院贝塞斯达国家心肺与血液研究所 Adrian Wiestner教授访谈

　　《肿瘤瞭望》：影响CLL预后的重要因素？

　　Prof. Wiestner: The most important factor is to separate Ig-mutated from Ig-unmutated CLL. This refers to sequences that encode the B-cell receptor. Surprisingly， it was found that CLL has both types， mutated and unmutated. This is part of the maturation process of B-cells and is something that is irreversible. Mutated in this regard is positive in the sense that they are more mature B-cells. These patients do better than those with Ig-unmutated cells. So that separates two important groups. The other important marker can be assessed by flow cytometry and that is CD49d expression.

　　Wiestner教授：影响CLL预后最重要的因素在于免疫球蛋白重链（IgVH）的突变状态，即编码B细胞受体的基因序列是否突变。可以将CLL分为突变型CLL（M-CLL）与未突变型CLL(U- CLL)。前者疾病进展快，生存期短；后者疾病进展慢，预后也较好。在某种层面上讲，IgVH突变具有积极的意义，这意味着有更多的成熟B细胞，患者的预后情况也更好。因此，IgVH突变是目前区别CLL预后的重要因素。此外，应用流式细胞技术评估CD49d的表达也被作为一项重要的预后指标。

　　《肿瘤瞭望》：CLL细胞遗传学的研究进展？

　　Prof. Wiestner: It may not be a single genetic event. One of the risk factors is a 17p deletion. One of the risk factors is a complex karyotype. One of the risk factors is NOTCH1 mutations in certain types of B-cell receptor specificities. There is a very elegant paper from Dr Ferreira’s group that does gene sequencing and they have identified some candidate mutations.

　　Wiestner 教授：CLL的发生发展过程并不是单一的遗传学改变，而是一系列复杂的基因改变的结果。其中，染色体 17P的缺失、复杂核型、B细胞受体特异型NOTCH1基因突变等都属于CLL的遗传学危险因素。目前，Ferreira博士的研究团队通过基因测序已确定了部分突变候选基因，这项研究成果有着的重大意义。