Ruiz-Delgado Guillermo J, Macías-Gallardo Julio, Lutz-Presno Julia, Garcés-Eisele Javier, Hernández-Arizpe Ana, Montes-Montiel Maryel, Ruiz-Argüelles Guillermo J
Centro de Hematología y Medicina Interna de Puebla, Clinica Ruiz, Puebla.
Rev Invest Clin. 2011 Jan-Feb;63(1):25-30.
Twenty one patients with CBF-AML presented prospectively in the Centro de Hematología y Medicina Interna de Puebla (Puebla, México) between February 1995 and March 2010, 14 with the t(8;21)(q22;q22) and 7 with the inv(16)(p13;q22)/t(16;16)(p13;q22); they represent 13% of all cases of AML. The median age of the patients was 24 years (range 1 to 61). Seven of 14 patients with t(8;21)(q22;q22) had an M2 morphology whereas 3/7 with the inv(16) had an M4 morphology; in addition to the myeloid markers identified by flow-cytometry (surface CD13, surface CD33, and cytoplasmic myeloperoxidase) lymphoid markers were identified in the blast cells of 8/14 cases of the t(8;21) patients, but in no patient with the inv(16). Nineteen patients were treated with combined chemotherapy and 16 (84%) achieved a complete molecular remission. Seven patients were auto or allografted. Relapses presented in 10/16 patients. The median probability of overall survival (OS) has not been reached being above 165 months, whereas the 165-month probability of OS and leukemia-free survival was 52%; despite a tendency for a better outcome of patients with the t(8;21), there were no significant differences in survival of patients with either the t(8;21) or the inv(16). In this single institution experience in México, we found that the CBF variants of AML have a similar prevalence as compared with Caucasian populations, that the co-expression of lymphoid markers in the blast cells was frequent in the t(8;21) and that these two AML subtypes were associated with a relatively good long-term prognosis. Further studies are needed to describe with more detail the precise biological features of these molecular subtypes of acute leukemia.
1995年2月至2010年3月期间，21例核心结合因子急性髓系白血病（CBF-AML）患者前瞻性地就诊于墨西哥普埃布拉市血液学与内科医学中心（Centro de Hematología y Medicina Interna de Puebla），其中14例为t(8;21)(q22;q22)，7例为inv(16)(p13;q22)/t(16;16)(p13;q22)；它们占所有急性髓系白血病病例的13%。患者的中位年龄为24岁（范围1至61岁）。14例t(8;21)(q22;q22)患者中有7例具有M2形态，而7例inv(16)患者中有3例具有M4形态；除了通过流式细胞术鉴定的髓系标志物（表面CD13、表面CD33和细胞质髓过氧化物酶）外，在14例t(8;21)患者中的8例原始细胞中还鉴定出了淋巴系标志物，但inv(16)患者中无一例出现。19例患者接受了联合化疗，16例（84%）实现了完全分子缓解。7例患者进行了自体或异体移植。16例患者中有10例出现复发。总生存期（OS）的中位概率尚未达到，超过165个月，而165个月时的OS和无白血病生存期概率为52%；尽管t(8;21)患者的预后有更好的趋势，但t(8;21)或inv(16)患者的生存期无显著差异。在墨西哥这个单一机构的经验中，我们发现AML的CBF变异型与白种人群的患病率相似，t(8;21)患者的原始细胞中淋巴系标志物的共表达很常见，并且这两种AML亚型的长期预后相对较好。需要进一步研究以更详细地描述这些急性白血病分子亚型的确切生物学特征。
