23例儿童T系急性淋巴细胞白血病的生物学特征分析

Biological characteristics of T-lineage acute lymphoblastic leukemia in 23 children

摘要:

目的：探讨儿童T细胞急性淋巴细胞白血病(T-ALL)的生物学性状及临床意义。方法：对初诊的23例儿童T-ALL患儿进行免疫分型、染色体、融合基因检测。结果：免疫分型中T系抗原以CD3阳性率最高，其次为CD7、CD5；B系抗原以CD19阳性率最高。23例中4例伴髓系抗原表达,阳性率达17%。CD34、HLA-DR表达阳性率分别为17%、22%。cCD3和cCD79的阳性表达率为100%和96%。8例行染色体检查,其中1例为超二倍体；1例为Ph+染色体。融合基因检测：5例中2例MLL基因重排异常，1例为SIL/TAL1阳性。CD3的表达与T-ALL首次化疗完全缓解率有关。结论：免疫分型分析是诊断T-ALL的重要手段, 但T-ALL免疫表型存在一定的异质性。对于儿童T-ALL的治疗预后评估，应结合细胞遗传学及分子遗传学进行综合判断。［中国当代儿科杂志，2010，12（8）：605-608］

Abstract:

OBJECTIVE: To investigate the biological characteristics of childhood T-lineage acute lymphoblastic leukemia (T-ALL) and their clinical significance. METHODS: Immunophenotyping was performed by three-color flow cytometry analysis using CD45 /SSC gating in 23 children with newly diagnosed T-ALL. Meanwhile cytogenetic analysis was performed. RESULTS: CD3+ expression of T-lineage antigens was apparently higher than CD7+ and CD5+ expression. CD19+ expression of B-lineage antigens was apparently higher than CD22+, CD10+ and CD20+ expression. Myeloid antigen was expressed in 4 cases (17%). CD34+ and HLA-DR+ were observed in 4 cases (17%) and 5 cases (22%), respectively. cCD3+ and cCD79+ were expressed in 23 cases (100%) and 22 cases (96%), respectively. The chromosome detection in 8 cases with T-ALL showed hyperdiploid or Ph+ chromosome (one case each). The fusion gene detection in 5 cases showed MLL rearrangements in two cases and positive SIL/TAL1 fusion gene in one case. CD3 expression was related with the complete remission rate. CONCLUSIONS: Immunophenotyping is an important tool for diagnosis of T-ALL. However, the immunophenotype of T-ALL is heterogeneous. So, immunophenotyping along with cytogenetic and molecular genetic analysis is needed in the treatment and prognosis evaluation of T-ALL.［Chin J Contemp Pediatr, 2010, 12 (8):605-608］