103例急性红白血病患者生物学特征与疗效观察

Biological Characteristics and Therapeutic Efficacy of 103 Patients with Acute Erythroleukemia

目的:本研究总结分析急性红白血病(AEL)的生物学特征与疗效。方法:回顾性分析2009年6月至2016年5月收治的103例急性红白血病患者肝功能、乳酸脱氢酶、凝血、形态学、免疫学、细胞遗传学及分子生物学特征,通过缓解率、复发率、无复发生存及总生存等指标观察疗效。结果:白细胞、粒细胞、血红蛋白及血小板中位数分别为3.04×10~9/L、0.67×10~9/L、66 g/L及45.0×10~9/L,71.1%的患者外周血涂片可以发现有核红细胞,无1例患者出现凝血功能异常,患者主要表达:CD13(93.5%)、CD117(89.1%)、HLA-DR(87.0%)、CD34(80.0%),部分患者表达淋系抗原CD4(42.9%)及CD7(28.9%)。82例患者核型分析显示,正常核型52.4%(43/82),异常核型41.5%(34/82),检查失败6.1%(5/82)。34例异常核型中,简单异常41.2%(14/34),复杂核型58.8%(20/34)。对60例患者检测融合基因显示,阳性率为16.7%(10/60)。27例患者检测预后基因突变显示,阳性率为77.8%(21/27)。初诊急性红白血病103例中接受化疗81例,可进行疗效分析66例。2个疗程累积缓解率45.5%(30/66),复发率36.7%(11/30),中位复发时间15.5(6.2-50)个月。可进行疗效分析66例患者中位生存时间为29个月。CR(30例)患者截至随访结束未达中位生存时间,明显优于未获CR(36例)患者12个月(P=0.001)。CR患者5年生存率65%,无复发生存(RFS)中位时间46.2个月,3年RFS为58%。结论:AEL患者具有高表达CD34抗原、多为复杂核型等生物学特点。虽然AEL患者缓解率低,生存期短,但缓解患者人群具有较好的长生存及良好的生存质量。

Objective： To investigate the biological characteristics and therapeutic efficacyt of acute erythroleukemia （AEL, AML-M6）. Methods： Blood cell count, liver function, lactate dehydrogenase level, coagulation, morphology, immunology, cell genetics and molecular biology were retrospectively analyzed in 103 cases of acute erythroleukemia patients admitted in our department from May 2016 to June 2009. The therapeutic efficacy was observed by means of remission rate, relapse rate, relapse-free survival and overall survival. Results： The medians of white blood cells, granulocyte, hemoglobin and platelet were 3. 04 x 109/L, 0. 67 x 109/L, 66 g/L, and 45 x 109/L, respectively. Nucleated red blood cells were found in the peripheral blood smears from 71.1% of AEL patients. None of the patients showed abnormal coagulation function. Flow cytometry analysis indicated that CD13 （93.5 % ）, CD117 （89. 1% ）, HLA-DR（ 87.0% ） , and CD34 （80.0%） were highly expressed in AEL, and lymphoid antigens of CD4 （42.9%）and CD7（28.9% ） were expressed in partial patients. Karyotype analysis in 82 patients showed 52.4% （43/82） normal karyotype, 41.5% （34/82） abnormal karyotype, and 6. 1% （5/82） failed tests. In the 34 cases with abnormal karyotype, there were 14 （41.2%） cases with simple chromosomal abnomality and 20 （58.8%） cases with complex karyotype. The positive rate of fusion gene accounted for 16.7% in 60 patients, and the gene mutations accounted for 77.8% in 27 patients. Among 103 cases of AEL, 81 cases were treated with chemotherapy, but 66 cases can be used for therapeutic analysis, as a results the total complete remission rate derived from 2 courses of treatment was 45.5 % （ 30/ 66）. The relapse rate was 36.7% （11/30）, and the median relapse time was 15.5 months （6.2 -50 months）. Themedian survival time of 66 patients for therapeutic analysis was 29 months. The median survival time of CR patients was very significantly longer than that of the non-CR patients（P = 0. 001 ）. The 5 year survival rate of CR patients was 65%, the median time of relapse-free survival （RFS） was 46.2 months and 3-years RFS was 58%. Conclusion： AEL is characterized by the highly expressed CD34 antigen, and complex karyotype. Although AEL has lower CR rate and poor prognosis, CR patients can achieve long-term survival and have good quality of life.