Philadelphia chromosome-positive acute lymphoblastic leukemia- current concepts and future perspectives.
Philadelphia chromosome-positive acute lymphoblastic leukemia- current concepts and future perspectives.
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DOI：
10.1046/j.1468-0734.2002.00066.x
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发表时间：
2002-06-01
期刊：
影响因子：
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通讯作者：
Kantarjian, Hagop M
中科院分区：
文献类型：
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作者：
Faderl, Stefan;Garcia-Manero, Guillermo;Kantarjian, Hagop M
费城染色体（Ph）阳性急性淋巴细胞白血病（ALL）在儿童中很少被诊断，但在成人ALL患者中构成最常见的细胞遗传学异常。与慢性粒细胞白血病（CML）不同，Ph阳性ALL患者通常表现为BCR-ABL蛋白的截短型p190 bcr-abl表达，不论年龄和断裂点的位置如何，Ph阳性ALL预后较差。虽然缓解率与Ph阴性ALL相同，但复发几乎是普遍的，长期生存仍然罕见。鉴于目前化疗巩固方案的不良结果，通常建议这些患者在首次缓解或尽快可行时进行干细胞移植。即使进行移植，对结果的影响也是有限的，迫切需要新的治疗概念。近年来最有希望的发展之一是酪氨酸激酶抑制剂如STI 571的引入。将提供当前Ph阳性ALL治疗模式的概述，并讨论新疗法的原理。
Philadelphia chromosome (Ph)-positive acute lymphoblastic leukemia (ALL) is diagnosed rarely in children, but constitutes the most frequent cytogenetic abnormality in adults with ALL. In contrast to chronic myeloid leukemia (CML), patients with Ph-positive ALL usually demonstrate expression of a truncated version of the BCR-ABL protein called p190bcr-abl. Irrespective of age and breakpoint location, Ph-positive ALL carries a poor prognosis. Although remission rates are identical to those of Ph-negative ALL, relapse is almost universal and long-term survival remains rare. Given the poor outcome with current chemotherapy consolidation programs, stem cell transplantation is usually recommended for these patients in first remission or as soon as feasible. Even with transplantation the impact on outcome is limited and new therapeutic concepts are urgently needed. One of the most promising developments in recent years has been the introduction of the tyrosine kinase inhibitors such as STI571. An overview of current treatment modalities in Ph-positive ALL will be provided and the rationale for new therapies will be discussed.