Getta Bartlomiej M, Devlin Sean, Park Jae H, Tallman Martin S, Berman Ellin
Leukemia Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Department of Epidemiology and Biostatistics, Memorial Sloan Kettering Cancer Center, New York, USA.
Leuk Res. 2018 Feb;65:80-85. doi: 10.1016/j.leukres.2017.12.008. Epub 2018 Jan 3.
Outcomes in very young CLL patients (age ≤40) are not well characterized. We compared 71 consecutive patients aged ≤40 with 142 "older" matched patients >40 from our institution and used SEER database as an independent comparison group. Patients in the two age groups were diagnosed at similar Rai stage. At diagnosis, very young patients had a similar rate of adverse cytogenetics, IGHV mutation and ZAP70 expression and had lower beta-2-microglobulin and a lower incidence of second malignancies. There was no difference between the groups with respect to incidence of autoimmune manifestations, family history of lymphoma, time to initiation of CLL therapy, response to therapy, or Richter's transformation. Variables including un-mutated IGHV and elevated LDH were associated with shorter times to treatment initiation in both groups. A trend to longer 5-year survival for very young patients in our institution (93% v 82%, p = 0.082) was validated by SEER data.
年龄非常小的慢性淋巴细胞白血病（CLL）患者（年龄≤40岁）的预后特征尚不明确。我们将本院71例连续入选的年龄≤40岁的患者与142例年龄＞40岁的“年长”匹配患者进行比较，并将监测、流行病学与最终结果（SEER）数据库作为独立的比较组。两个年龄组的患者在相似的Rai分期时被诊断。诊断时，年龄非常小的患者不良细胞遗传学、免疫球蛋白重链可变区（IGHV）突变及ζ链相关蛋白激酶70（ZAP70）表达率相似，且β2微球蛋白水平较低，第二原发恶性肿瘤发生率较低。两组在自身免疫表现发生率、淋巴瘤家族史、开始CLL治疗的时间、对治疗的反应或Richter转化方面无差异。包括未突变的IGHV和乳酸脱氢酶（LDH）升高在内的变量与两组开始治疗的时间较短相关。本院年龄非常小的患者5年生存率有延长趋势（93%对82%，p = 0.082），这一趋势得到了SEER数据的验证。
