髓系抗原阳性儿童急性淋巴细胞白血病临床特点及预后

Childhood acute lymphoblastic leukemia with myeloid antigen expression and its prognosis

目的分析髓系抗原阳性儿童急性淋巴细胞白血病(ALL)的临床特点及预后关系。方法根据国际白血病欧洲协作组(EGIL)标准将1999—2004年上海交通大学医学院附属上海儿童医学中心收治的33例髓系抗原阳性表达的ALL(My+ALL)分为双表型、双系列型给予正规治疗。对其预后进行观察。结果(1)My+ALL患儿,双表型26例(78.8%,26/33),其中B系ALL伴髓系表达17例(65.4%,17/26),T系ALL伴髓系表达6例(23.1%,6/26),T系B系伴髓系表达3例(11.5%,3/26)。双系列ALL患儿7例(21.2%,7/33)。(2)26例双表型ALL患儿治疗35d,缓解率80.7%。7例双系列型ALL仅1例达缓解(14.3%)。(3)生存状态双表型26例中20例处于缓解状态(76.9%),双系列型7例中仅1例(14.3%)。(4)复发情况双表型6例复发(23.1%,6/26),双系列7例中6例复发(85.7%)。结论髓系抗原阳性表达在儿童ALL时不能作为预后不良的因素,但双系列白血病患儿预后差,复发率高,长期生存机会少。

Objective To analyse the clinical characteristics and their relationship with the prognosis in childhood acute lymphoblastic leukemia with myeloid antigen expression. Methods Thirty-three children with acute lymphoblastic leukemia with myeloid antigen expression （My＋ ALL） at the same time were subclassified as double antigenic expression and double lineage type in accordance with the criteria formulated by the European Collaboration Study Group of the International Leukemia （EGIL） and standardized treatment protocols were given. Results （ 1 ） Twenty-six children had double expression （78. 8% ） in My^＋ ALL of them, 17 cases belonged to the B lineage ALL with Myeloid antigen expression （ 65.4% ）. Six cases with T lineage ALL had Myeloid expression （ 23. 1% ）. Three with T lineage and B lineage had myeloid antigen expression at the same time （ 11.5% ） whereas 7 cases belonged to the double lineage ALL （ 21.2% ）. （2） Twenty-six children with double antigenic expression ALL received standardized treatment protocols with complete remission rate of 76. 9% in 35 days,whereas only one case achieved CR（ 14. 3% ） in 7 children with double lineage ALL. （3） Survival status：1 8 of 26 children with double antigenic expression ALL were still in CR （65 % ） while.only 1 of the 7 children with double lineage ALL remained in the CR（ 14. 3% ）. （4） Relapse rate： 6 children with double antigenic expression ALL relapsed （ 23. 1% ） while 6 of 7children with double lineage ALL relapsed （ 85.7% ）. Conclusion Myeloid antigen positive expression in children with ALL could be considered as poor prognostic factor, but the prognosis for children with double lineage ALL is very poor with high relapse rate and few long-term survival frequency.