Abstract:

Objective　To analyze the clinical characteristics and prognosis of pediatric acute T lymphoblastic leukemia. 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 relapsed 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 significantly 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.

Key words: child, acute T lymphoblastic leukemia, relapse, prognosis

摘要：

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（IgH）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≥ 40 μmol/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IgH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。

关键词: 儿童, 急性T淋巴细胞白血病, 复发, 预后