儿童急性T淋巴细胞白血病临床疗效及预后相关因素73例分析

Clinical outcome of seventy-three cases of pediatric acute T lymphoblastic leukemia and analysis of prognosis factors

目的总结儿童急性T淋巴细胞白血病(T-ALL)的临床特点及疗效,分析其预后影响因素。方法回顾性总结自2003年3月至2014年3月北京大学人民医院收治的73例初治T-ALL及累及骨髓的T淋巴母细胞淋巴瘤患儿的病例资料及随访资料,分析预后相关因素。结果 73例患儿中25例患儿复发,3例因化疗或移植并发症死亡,5年累积复发率(CRF)为(37.89±6.02)%,5年无事件存活率(EFS)为(58.74±6.01)%。初诊时年龄≥13岁的患儿5年EFS显著减低,脾肋下≥9 cm及初诊时免疫球蛋白重链(Ig H)基因重排阳性患儿的5年CRF显著升高。大剂量甲氨蝶呤(MTX)化疗中,MTX血药浓度峰值≥40μmol/L患儿的预后明显改善。结论儿童T-ALL的长期疗效仍不令人满意。初诊时年龄≥13岁、脾肋下≥9 cm提示预后较差。初诊时Ig H基因重排阳性患儿可能存在较高复发风险。大剂量MTX的治疗可使T-ALL患儿受益。

Objective To analyze the clinical characteristics and prognosis of pediatric acute T lymphoblastic leuke- mia. Methods Clinical characteristics of 73 children with acute T lymphoblastic leukemia and T-cell lymphoblastic lymphoma involving the bone marrow treated in Peking University People＇ s Hospital from March 2003 to March 2014 were retrospectively analysed, and the prognosis factors were analyzed. Results Of the 73 patients, 25 children re- lapsed and 3 died from complications during chemotherapy or transplantation. Their 5-year CRF was （37.89±6.02）% and 5-year EFS was （58.74±6.01）%. The 5-year EFS decreased significantly when the initial onset age was more than 13. The 5-year CRF increased significantly when the initial onset size of spleen was bigger than 9 cm under the costal margin or the IgH rearrangement was positive. The 5-year CRF decreased significantly and 5-year EFS increased signifi- cantly when average peak methotrexate （MTX） blood concentration was more than 40 μmol/L during high-dose MTX chemotherapy. Conclusion The treatment result of children with T-ALL is unsatisfactory. Initial onset age ≥13 and huge spleen indicate poor prognosis. Positive IgH rearrangement may be associated with high risk of recurrence. T-ALL patients can benefit from high-dose MTX therapy.