摘要
目的探讨婴儿急性白血病（IAL）的临床表现、细胞遗传学及分子生物学检查特点。方法对2005年1月-2009年12月华中科技大学同济医学院附属同济医院和武汉市儿童医院初诊的30例IAL患儿临床表现、实验室检查、细胞形态学、免疫学、细胞遗传学和分子遗传学分型检查及随访资料进行回顾性分析。结果临床表现为发热14例,皮下出血5例,面色苍白、面色苍黄各4例。全身浅表淋巴结大5例,骨质破坏5例,肝和（或）脾超过肋下4 cm 17例,中枢神经系统浸润1例。发病至就诊时间2~75 d。WBC计数为114.78（1.06~681.37）×109L-1,Hb 67（38~101）g.L-1,PLT 61（1~355）×109L-1。IAL 30例外周血涂片均可见幼稚细胞。30例中ALL 15例,急性非淋巴细胞白血病（ANLL）14例,分类不明1例。12例行免疫分型检查,5例ALL均为B系,其中CD10表达4例,CD7与CD15共同表达1例;7例ANLL中CD19表达2例。12例染色体检查结果异常者3例。13例IAL行融合基因检测,结果E2A/PBX1、BCR/ABL阳性各1例。30例中仅4例ALL接受化疗,但最终均放弃治疗。而对未治疗的26例1个月后电话随访均死亡。结论 IAL的临床表现缺乏特异性;ALL及ANLL均可发生,且比率相当;ALL中以B系多见;IAL常伴染色体异常。但由于绝大多数未得到有效化疗而放弃治疗,故IAL的预后较年长儿差。
Objective To explore the clinical,cytogenetic and biological characteristics of infantile acute leukemia（IAL）.Methods From Jan.2005 to Dec.2009,in the Department of Pediatrics,Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology,and Wuhan Children′s Hospital,clinical manifestations and laboratory findings,cytomorphdogy,immunology,cytogenetics,molecular genotype and follow-up data were reviewed retrospedively in 30 cases of IAL.Results There were 14 cases with fever,5 cases with subcutaneous hemorrhage,4 cases with sallow complexion,4 cases with pale complexion.Five cases with superficial lymphadenopathy,5 cases with bone lesion,17 cases with hepatosplenomegaly,1 case with central nervous system infiltration.The duration between attack and treatment was from 2-75 days.White blood cell count was 114.78（1.06-681.37） ×109 L-1,hemoglobin was 67（38-101） g·L-1,platelet was 61（1-355）×109 L-1.Periphery blasts were identified on all 30 cases.Of the 30 cases,15 cases were acute lymphocytic leukemia（ALL）,14 cases were acute nonlymphocytic leukemia（ANLL）,and 1 case had unknown causes.Twelve cases were acquired morphology,immunology,cytogenetics and molecular typing.Five cases of ALL were diagnosed as B-ALL,with 4 cases of CD10 positive,1 case of CD7 and CD15 co-expression,2 cases out of 7 ANLL cases were found CD19 expression.Three cases out of 12 cases showed chromosome abnormalities.Among 13 cases with fusion gene assay,1 case was E2A/PBX1 positive,another case was BCR/ABL positive.Four of 30 cases accepted chemotherapy,but eventually gave up.Another 26 untreated cases were confirmed dead after 1 month.Conclusions IAL has no unique clinical features.ALL and ANLL would be the approximate proportion;B-cell type is the most immunophenotype of ALL.Abnormal chromosomes are found frequently.The prognosis is poor since most of the patients gave up chemotherapy after diagnosis.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2011年第3期177-179,共3页
Journal of Applied Clinical Pediatrics
