11q23异常儿童急性白血病的临床及实验室分析

目的　研究伴 11q2 3异常儿童急性白血病 (AL)的形态学、免疫学、细胞遗传学与其临床特征和预后的关系。方法　对 32 0例AL中 18例伴有 11q2 3异常的患儿进行回顾性分析 ,包括细胞形态学观察、流式细胞仪细胞免疫表型检测、R带核型分析和临床预后。以同期核型正常的 2 0例AL患儿作为对照。结果　 18例伴 11q2 3异常AL患儿中 14例为急性淋巴细胞白血病 (ALL) ,4例为急性髓系白血病 ,其中M5b3例 ,M2a1例。进行免疫表型分析的 16例患儿中 ,13例有淋系抗原表达 ,3例除表达髓系抗原外均显示CD34 阳性。异常核型有 6种 :t(4 ;11) (q2 1;q2 3) 6例 ;t(10 ;11) (p13;q2 3) 3例 ;t(11;19) (q2 3;p13) 1例 ;t(11;17) (q2 3;q11) 1例 ;t(8;11) (q2 3;q2 3) 1例 ;del(11) (q2 3) 6例。本组AL完全缓解 (CR)率为 72 .2 % ,与对照组的 80 .0 %相比 ,差异无显著性 (P >0 .0 5 ) ,而死亡率为 6 1.1% ,与对照组的 2 5 .0 %相比 ,差异有显著性 (P <0 .0 5 )。结论　 11q2 3异常主要见于儿童ALL和急性单核细胞白血病 ,具有较为独特的临床、血液学和预后特点。

Clinical and laboratory studies on childhood acute leukemia with 11q23 abnormalities

Objective To investigate the interrelations among morphology, immunology, cytogenetics and clinical outcome in childhood acute leukemia with 11q23 abnormalities. Methods Eighteen patients with 11q23 abnormalities, from 320 childhood acute leukemia patients,were retrospectively analysed for cell morphology,flow cytometry, immunophenotyping,R banding karyotype as well as clinical features and prognosis.Twenty cases of childhood AL with normal karyotype during the same period were used as control. Results The incidence of 11q23 abnormalities in our childhood acute leukemia patients was 5.63% including 14 acute lymphoblastic leukemia (ALL) and 4 acute myeloid leukemia(AML). Of 16 cases immunophenotypically tested, 13 expressed lymphoid antigens and 3 CD 34 and other myeloid antigens. Karyotype analysis disclosed the following abnormalities: t(4;11)(q21;q23) in 6 cases , t(10;11)(p13;q23) in 3, t(11;19)(q23;p13) in one and del(11)(q23) in 6. The complete remission rate for these patients with 11q23 abnormalities was comparable to that of the control (72.2% vs 80 0%,P>0.05),while the mortality rate in the former was significantly higher than that in the latter (61.1% vs 25 0%,P<0.05). Conclusions 11q23 abnormalities were mainly seen in childhood ALL and acute monocytic leukemia with unique prognostic features.