儿童急性早幼粒细胞白血病的临床研究

目的 　探讨儿童急性早幼粒细胞白血病 (APL)的临床及实验室特征。方法对 4 1例APL初治患儿进行临床分析 ,男 2 3例 ,女 1 8例 ,中位年龄 1 2 ( 3～ 1 8)岁。 2 7例进行了遗传学和PML/RARα融合基因检测。 38例患儿进行了免疫分型。诱导及维持治疗全部应用维甲酸 ;采用HA/D/MA方案强化治疗。结果 　临床及实验室特征 :贫血 31 /41例 ,出血 2 3/41例 ,发热 1 9/41例 ,肝肿大 4 /41例 ,脾肿大 3/41例 ,淋巴结肿大 6 /41例 ;Hb 73( 30～ 1 1 0 )g/L ;WBC 2 9( 1 1～ 90 )× 1 0 9/L ;BPC 4 0 ( 1 4～ 1 2 5)× 1 0 9/L。 2 7例进行细胞遗传学分析 ,t( 1 5;1 7) (q2 2 ,q1 2 )者 1 7例 ,同时检测PML/RARα融合基因 1 6例为阳性 :1 0例为正常核型 ,而PML/RARα融合基因 1 0例均为阳性。 38例患儿免疫分型共同特征表现为CD9、CD13 、CD3 3 高表达 ,而HLA -DR及CD3 4为阴性。临床疗效 :维甲酸治疗的完全缓解 (CR)率为 89 76 % ( 36 /41 )。 3年无复发生存率 95% ( 1 9/2 0 )。结论 　APL的临床表现以贫血、出血、发热为主要特征。免疫分型的共同特征为CD9、CD13 、CD3 3 高表达 ,而HLA -DR及CD3 4为阴性。APL的形态学诊断与PML/Rarα融合基因及染色体的符合率分别为 96 3% ( 2 6 /2 7)和 6 3% ( 1 7/2 7)。?

A clinical study on childhood with acute promyelocytic leukemia

Objective To evaluate the clinical features, cytogenetics and outcome in childhood acute promyelocytic leukemia(APL). Methods 41 patients with acute promyelocytic leukemia were studied retrospectively. The median age at diagnosis was 12(3～18) years old. Induction and maintenance therapy only used retinoic acid. Intensive therapy consisted of cytosine arabinoside and an anthracycline, with or without other agents.Result Hepatomegaly, splenomegaly and lymphadenopathy were observed in 9.8%, 7.6%, and 14.6% of the cases, respectively. The median WBC count was 2.9(1.1～90)×10 9/L. Anemia(hemoglobin<80 g/L) and thrombocytopenia (<40×10 9/L) were present in more than half of the patients. Identification of Immunophenotype subtypes of APL in 38 patients, it is characterized by the high express CD_9? CD_ 13 and CD_ 33 . Cytogenetic studies demonstrated the characteristic 15;17 translocation in about 63% of the patients analyzed, PML/RAR α positive cases was 96.3% of the patients analyzed. Thirty-six patients (89.76%) achieved complete remission (CR) while early fatal hemorrhage was the predominant cause of induction failure. The 3 years event-free survival rates was 95% (19/20). Conclusion Childhood APL were characterized by anemia, hemorrhage, and fever. PML/RAR α positive cases was 96.3% of the patients analyzed, chromosome 15;17 translocation in about 63% of the patients analyzed. Immunophenotype were characterized by the high express CD_9? CD_ 13 and CD_ 33 . These results indicate that APL patients receiving ATRA plus anthracycline-based chemotherapy appear to be effectively.