老年人急性淋巴细胞白血病的特点及预后

Characteristics and clinical outcomes of elderly patients with acute lymphoblastic leukemia

目的分析老年急性淋巴细胞白血病（ALL）患者同非老年成年ALL患者临床特征及分子遗传学等方面的差异，进一步探讨老年ALL患者不良预后因子。 方法将收治的279例ALL患者分为老年组（60~79岁）及非老年组（14~59岁），分析两组患者的临床特征、实验室指标及相关基因IKZF1、PAX5、NOTCH1、PHF6、SH2B3、LEF1、JAK1的分子遗传学异常，并分析这些指标与临床预后的关系。 结果老年组男性患者比例低于非老年组[42.9%（21/49）比61.7%（142/230），P=0.015）。老年组B-ALL发生率、费城染色体阳性（Ph＋）率及髓系标记CD33阳性率均明显高于非老年组[87.8%（43/49）比70.4%（162/230），P=0.009；47.8%（22/49）比27.4%（58/230），P=0.007；56.8%（21/49）比39.0%（64/230），P=0.049]。老年组ALL患者的淋巴结肿大发生率、总完全缓解（CR）率低于非老年组[20.0%（9/49）比38.9%（81/230），P=0.016；68.3%（28/41）比91.3%（178/195），P〈0.001]。老年组ALL患者3、6、12、24个月总生存（OS）率均低于非老年组（64.6%比84.4%，P=0.001；50.0%比73.8%，P=0.001；29.2%比52.4%，P=0.003；6.2%比26.2%，P=0.003）。两组患者的PAX5、NOTCH1、PHF6、SH2B3、LEF1、JAK1基因突变率差异均无统计学意义（均P〉0.05）。 结论老年ALL患者临床及实验室指标同非老年成年ALL患者存在差异，老年组预后更差，需要个体化治疗，以改善预后。

ObjectiveTo explore the differences in clinical and laboratory parameters between elderly and non-elderly patients with acute lymphoblastic leukemia （ALL）. Poor prognostic factors in elderly patients were explored to guide the individualized treatment. MethodsTwo hundred and seventy-nine ALL patients were divided into two groups： elderly group with their age more than 60 years （60-79 years） and non-elderly group with their age less than 60 years （14-59 years）. The differences in clinical and laboratory parameters, abnormal molecular genetics on related genes, including IKZF1, PAX5, NOTCH1, PHF6, SH2B3, LEF1, and JAK1, as well as the correlations with treatment response and clinical outcome were compared between the two groups. ResultsMales accounted for a smaller part in elderly group [42.9% （21/49） vs. 61.7%（142/230）, P=0.015]. The percentage of B cell lineage ALL （B-ALL）, Philadelphia chromosome positive （Ph ＋） and CD33 positive rate were higher in elderly group compared with those in non-elderly group [87.8% （43/49） vs. 70.4% （162/230）, P=0.009; 47.8% （22/49） vs. 27.4% （58/230）, P=0.007; 56.8% （21/49） vs. 39.0% （64/230）, P=0.049, respectively]. While both lymphodenopathy and total complete remission （CR） rate gained the upper hand in non-elderly group [38.9% （81/230） vs. 20.0% （9/49）, P=0.016; 91.3% （178/195） vs. 68.3% （28/41）, P〈0.001, respectively]. Moreover, elderly group had lower 3-month, 6-month, 12-month and 24-month overall survival （OS） rates （64.6% vs. 84.4%, P=0.001; 50.0% vs. 73.8%, P=0.001; 29.2% vs. 52.4%, P=0.003; 6.2% vs. 26.2%, P=0.003, respectively） than those of non-elderly group. No significant differences in mutation rates of PAX5, NOTCH1, PHF6, SH2B3, LEF1 and JAK1 were found （all P〉0.05）. ConclusionsCompared with non-elderly ALL patients, elderly ones harbor their intrinsic characteristics which might give rise to inferior outcomes. As a consequence, more attention should be poured into treating this particular group of ALL patients to improve their prognosis.