染色体t(4;11)急性淋巴细胞白血病——10例临床和实验室分析

Chromosome t(4;11) acute lymphoblastic leukemia: an analysis of 10 cases

目的 研究t(4 ;1 1 )急性淋巴细胞白血病 (急淋 )的形态学、免疫学、细胞遗传学和临床特点。方法 1 0例t(4 ;1 1 )急淋 ,其中 8例为初诊患者 ,2例为急淋复发患者 ,经DOPL(柔红霉素、长春新碱、泼尼松、左旋门冬酰胺酶 )、VP(长春新碱、泼尼松 )等不同联合方案化疗。 7例采用流式细胞仪进行细胞免疫标记分析 ;所有病例采用骨髓细胞直接法或短期培养法制备染色体标本 (例 7为外周血培养 ) ,采用RHG显带技术进行核型分析。结果 t(4 ;1 1 )急淋占同期急淋的 3 .5%。 1 0例t(4 ;1 1 )急淋中 6例WBC >1 0 0× 1 0 9/L ,1 0例均有不同程度的肝、脾和淋巴结浸润。 5例为单纯t(4 ;1 1 ) ,5例同时出现其它的染色体异常。细胞免疫学表面标记除 1例为HLA DR和CD5 阳性外 ,6例CD19阳性 ,5例CD2 2阳性 ,支持绝大多数为B祖细胞急淋。本组急淋患者中位生存期为 5.5个月。结论 t(4 ;1 1 )急淋是一种预后不良的、独特的临床细胞遗传学亚型。

Objective To characterize morphologically, immunophenotypically, cytogenetically and clinically the acute lymphoblastic leukemia (ALL) with t(4;11)(q21;q23). Methods Ten cases of t(4;11) ALL were reported. Of them, eight patients were de novo and two relapsed. The patients were treated with combination chemotherapy of DOPL or VP regimen. Immunophenotypic analysis was performed by flow cytometry in seven cases. Cytogenetic analysis was performed using bone marrow cells prepared directly and/or after 24h culture (case 7 using peripheral blood). RHG banding was used for karyotypic analysis. Results Ten (3.5%) of 285 ALL patients were found to be t(4;11) ALL. In these 10 patients, increased WBC(>100×10 9/L) was found in 6 cases. Liver, spleen and/or lymph nodes were involved in all. t(4;11) was detected as a single abnormality in 5 karyotypes whereas the other 5 showed additional aberration besides t(4;11). Six patients were CD 19 (+),five were CD 22 (+) and one was HLA DR(+),CD 5(+), supporting that most cases of t(4;11) ALL were B cell origin. Median survival time was 5.5 months in the present series. Conclusion t(4;11) ALL was a subtype with unique clinical and cytogenetic features, and had a poor prognosis.