Frequency and clinical significance of the MLL gene rearrangements in infant acute leukemia.
Frequency and clinical significance of the MLL gene rearrangements in infant acute leukemia.
复制标题
婴儿急性白血病 MLL 基因重排的频率和临床意义。
作者：
T. Taki;K. Ida;Fumio Bessho;Ryoji Hanada;Akira Kikuchi;Katsuya Yamamoto;Masahiro Sako;Masahiro Tsuchida;Masao Seto;Ryuzo Ueda;Y. Hayashi
本文分析了42例婴儿急性白血病（包括37例急性淋巴细胞白血病（ALL）和5例急性髓细胞白血病（AML））MLL基因重排的频率和临床意义。在37例ALL病例中有27例（73%）和所有5例AML病例中发现了MLL基因重排。27例MLL基因重排的ALL中24例11 q23异常。MLL基因重排与CD 10表达缺失和预后不良显著相关，但与6个月以下的年龄、高白细胞血症、髓系相关抗原表达或CNS白血病无关。MLL基因重排的ALL病例的3年总生存率为5.3 +/-5.2%，而生殖系MLL病例为88.9 +/-10.5%（P=0.0001）。CD 10表达缺失也与预后不良相关（9.9 +/-6.6% vs 85.7 +/-13.2%，P = 0.0003）。在5例AML病例中，3例存活了27个月至67个月。这些结果表明，婴儿ALL伴MLL基因重排与不良预后密切相关。我们认为，婴儿ALL应根据MLL基因重排的存在与否，采用不同的化疗方案。
We have analyzed the frequency and clinical significance of the MLL gene rearrangements in 42 cases of infant acute leukemias; including 37 cases of acute lymphoblastic leukemia (ALL) and five cases of acute myeloid leukemia (AML). MLL gene rearrangements were found in 27 of the 37 ALL cases (73 percent), and in all five AML cases. Cytogenetic studies showed 11q23 abnormalities in 24 of 27 ALL cases with MLL gene rearrangements. MLL gene rearrangements were significantly correlated with absence of CD10 expression and poor prognosis, but not with age under 6 months, hyperleukocytosis, myeloid-associated antigen expression, or CNS leukemia. The 3-year overall survival rate for ALL cases with MLL gene rearrangements was 5.3 +/- 5.2 percent, compared with 88.9 +/- 10.5 percent for cases with germline MLL (P=0.0001). Absence of CD10 expression was also associated with poor prognosis (9.9 +/- 6.6 percent vs 85.7 +/- 13.2 percent, P = 0.0003). Of the five AML cases, three have remained alive for 27 months to 67 months. These findings suggest that infant ALL with MLL gene rearrangement is strongly associated with poor prognosis. We consider that infant ALL should be treated on different chemotherapy protocols according to the presence or absence of MLL gene rearrangement.