ZAP-70 compared with immunoglobulin heavy-chain gene mutation status as a predictor of disease progression in chronic lymphocytic leukemia
ZAP-70 compared with immunoglobulin heavy-chain gene mutation status as a predictor of disease progression in chronic lymphocytic leukemia
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DOI：
10.1056/nejmoa040857
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发表时间：
2004-08-26
影响因子：
158.5
通讯作者：
Kipps, TJ
中科院分区：
文献类型：
--
作者：
Rassenti, LZ;Huynh, L;Kipps, TJ
背景：慢性淋巴细胞白血病（CLL）的病程是多变的。在侵袭性疾病中，CLL细胞通常表达未突变的免疫球蛋白重链可变区基因（IgV（亚H））和70-kD ζ相关蛋白（ZAP-70），而在惰性疾病中，CLL细胞通常表达突变的IgV（亚H），但缺乏ZAP-70的表达。方法：我们对307例CLL患者的CLL B细胞进行了ZAP-70和重排IgV（亚H）基因突变的检测。然后我们调查了结果与从诊断到初始治疗的时间之间的关系。结果：我们发现，在164例IgV（亚H）基因未突变的患者中，有117例（71%）的ZAP-70表达高于定义的阈值水平，但在143例IgV（亚H）基因突变的患者中，只有24例(17%，P
BACKGROUND:The course of chronic lymphocytic leukemia (CLL) is variable. In aggressive disease, the CLL cells usually express an unmutated immunoglobulin heavy-chain variable-region gene (IgV(sub H)) and the 70-kD zeta-associated protein (ZAP-70), whereas in indolent disease, the CLL cells usually express mutated IgV(sub H) but lack expression of ZAP-70.METHODS:We evaluated the CLL B cells from 307 patients with CLL for ZAP-70 and mutations in the rearranged IgV(sub H) gene. We then investigated the association between the results and the time from diagnosis to initial therapy.RESULTS:We found that ZAP-70 was expressed above a defined threshold level in 117 of the 164 patients with an unmutated IgV(sub H) gene (71 percent), but in only 24 of the 143 patients with a mutated IgV(sub H) gene (17 percent, P