Guerrasio A, Rosso C, Martinelli G, Lo Coco F, Pampinella M, Santoro A, Lanza C, Allione B, Resegotti L, Saglio G
Dipartimento di Scienze Biomediche ed Oncologia Umana, CNR-CIOS, Ospedale S. Luigi Gonzaga, Università di Torino, Italy.
Br J Haematol. 1995 Jun;90(2):364-8. doi: 10.1111/j.1365-2141.1995.tb05160.x.
The t(8;21) (q22;q22) translocation is a recurring chromosomal abnormality observed in about 20-40% of AML patients with subtype FAB M2 (AML-M2). The molecular facet of this translocation is represented by the formation of a new hybrid gene, the AML1-ETO, which is regularly transcribed in a chimaeric mRNA and translated into a new fusion protein believed to have a key role in the pathogenesis of this type of leukaemia. We looked for the presence of AML1-ETO transcripts, by RT-PCR, in 49 unselected patients affected by AML-M2 diagnosed at various Italian Institutions. A hybrid transcript was detected in 11 cases (23%). Minimal residual disease status was investigated in three patients in continuous complete remission (CCR) after a median follow-up of 44 months; at least one sample from each subject was found positive for the AML1-ETO transcript suggesting a long-term persistence of t(8;21) leukaemic cells. In two female patients in CCR a 'clonality' analysis was performed on peripheral blood DNA by exploiting the X chromosome inactivation pattern of the human androgen-receptor gene (HUMARA); in both cases the results were consistent with the presence of a polyclonal haemopoiesis. Our data confirm that the persistence of residual cells expressing the AML1-ETO transcripts is a frequent occurrence even in patients with long-term remission; on the other hand, clonality assays indicate that in t(8;21) leukaemias long-term remission haemopoiesis is sustained by a polyclonal bone marrow reconstitution.
t(8;21)(q22;q22)易位是一种常见的染色体异常,在约20%-40%的FAB M2亚型急性髓系白血病(AML-M2)患者中可见。这种易位的分子层面表现为形成一种新的融合基因AML1-ETO,它经常转录成嵌合mRNA并翻译成一种新的融合蛋白,据信该蛋白在这类白血病的发病机制中起关键作用。我们通过逆转录聚合酶链反应(RT-PCR),在意大利不同机构诊断的49例未经选择的AML-M2患者中寻找AML1-ETO转录本的存在情况。在11例患者(23%)中检测到了杂交转录本。对3例持续完全缓解(CCR)的患者进行了微小残留病状态调查,中位随访44个月;发现每个受试者至少有一个样本的AML1-ETO转录本呈阳性,提示t(8;21)白血病细胞长期持续存在。对2例处于CCR的女性患者,利用人类雄激素受体基因(HUMARA)的X染色体失活模式对外周血DNA进行了“克隆性”分析;在这两个病例中,结果均与多克隆造血的存在一致。我们的数据证实,即使在长期缓解的患者中,表达AML1-ETO转录本的残留细胞持续存在也很常见;另一方面,克隆性分析表明,在t(8;21)白血病中,长期缓解的造血是由多克隆骨髓重建维持的。