CAJ | 학술논문

目的总结2岁以下婴幼儿急性淋巴细胞白血病(ALL)的生物学特征.方法回顾性分析1996年1月至2005年12月在北京儿童医院诊治的2岁以下ALL患儿的临床资料及治疗效果,随访至2006年8月31日.结果共41例患儿,占同期ALL的3.9%.诊断后治疗者28例.诊断时年龄≤12个月11例,白细胞数大于20×109/L 21例,中枢神经系统侵犯者5例,骨髓免疫学均为B细胞来源,其中带有髓系标志22例,17例存在染色体易位,其中7例为11q23易位形成的融合基因.诊断后治疗的患儿.于诱导治疗第28天骨髓完全缓解率100%,复发4例,复发时间为缓解后9～34个月(中位14个月),4例复发患儿均为高白细胞,且3例具有11q23形成的融合基因.22/28例患儿生存并随访至今,随访时间8～124个月(中位39个月);持续完全缓解时间1～124个月(中位36.5个月).结论 2岁以下婴幼儿ALL的生物学特点明显,临床多表现为高白细胞,骨髓免疫学以B细胞型为主,且髓系相关抗原联合表达的频率很高,染色体以11q23易位形成的融合基因为多见,骨髓复发仍然是治疗失败的重要原因,伴有11q23易位形成的融合基因者预后差.
목적 총결2세이하영유인급성림파세포백혈병(ALL)적생물학특정.방법 회고성분석1996년1월지2005년12월재북경인동의원진치적2세이하ALL환인적림상자료급치료효과,수방지2006년8월31일.결과공41례환인,점동기ALL적3.9%.진단후치료자28례.진단시년령≤12개월11례,백세포수대우20×109/L 21례,중추신경계통침범자5례,골수면역학균위B세포래원,기중대유수계표지22례,17례존재염색체역위,기중7례위11q23역위형성적융합기인.진단후치료적환인.우유도치료제28천골수완전완해솔100%,복발4례,복발시간위완해후9～34개월(중위14개월),4례복발환인균위고백세포,차3례구유11q23형성적융합기인.22/28례환인생존병수방지금,수방시간8～124개월(중위39개월);지속완전완해시간1～124개월(중위36.5개월).결론 2세이하영유인ALL적생물학특점명현,림상다표현위고백세포,골수면역학이B세포형위주,차수계상관항원연합표체적빈솔흔고,염색체이11q23역위형성적융합기인위다견,골수복발잉연시치료실패적중요원인,반유11q23역위형성적융합기인자예후차.
Objective Repots of the clinical feature,immunophenotype and cytogenetic trait for children at age of less than 2 year old with newly diagnosed acute lymphoblastic leukemia(AEL).Methods Between 1996.1～2005.12 in Beijing Children's Hospital. children less than 2 year old with ALL were enrolled.Using retrospective methods,we collected and studied the clinical data,following up till to August 31.2006.Results There were 41 children with ALL at age of less than 2 year old.accounting for 3.9% of all in the same time,including 11 for the age less than 12 months old,21 with WBC more than 20×109/L 5 with center nervous system(CNS)leukemia.All cases are B-cell immunophenotype,22 with immunophenotypic co-expression of myeloid associated antigens,17 cytogenetic abnormalities and fusion gene positive,among them, 7 involving ALL-1/MLL 11q23 gene rearrangements.Among 41 cases.28 cases were given combined intensive chemotherapy and achieved complete remission(CR)with induction therapy for 28 days,4 cases were relapsed from CR 9～34 months(medium 18 months).22 cases were follow-up continuously during 8 to 124 months(medium 39 months),continue complete remission(CCR)from 1 to 124 months(medium36.5 months).Conclusion Children with ALL at the age less than 2 year old display unique biological features as compared with older children.is associated with a hish leukocyte count at presentation and CNS involvement. The immunophenotype is usually B-lineage and is characterized by the co-expression of myeloid-associated antigens. and presence of involving ALL-1/MLL 11q23 gene rearrangements. Marrow relapse remains the primary mode of failure. Cytogenetic abnormalities involving ALL-1/MLL 11q23 gene rearrangements with ALL have a very poor outcome.