急性红白血病13例临床特点及预后分析

本研究旨在分析急性红白血病的临床特征及其预后.回顾性分析2007年10月到2012年10月共收治305例急性白血病患者中13例急性红白血病患者的临床特征,及其形态学,免疫学、细胞遗传学和分子生物学检查结果.结果表明,红系及非红系同时表达增高,髓系抗原主要表达CD13、CD33、CD117、CD34,部分有核红系抗原表达Gly、CD71.核型分析显示3例有轻微核型异常,2例有复杂核型,1例为正常核型,在其余患者未检测.分子生物学检测发现,5例存在预后不良基因,其中3例MLL-MLL融合基因阳性,1例MLL突变,1例为NRAS基因突变,在其余8例中未检测出异常基因.经含地西他滨诱导化疗方案治疗,患者CR率为53.8％ （7/13）,PR率为15.4％ （2/13）,最终8例患者行异基因造血干细胞移植.3年OS率79％,3年RFS率78％.结论：AML-M6为急性白血病中特殊亚型,由MDS转化,含有不良基因,预后较差,采用异基因造血干细胞移植及DAC相关化疗方案可以提高生存率.

Analysis of Clinical Characteristics and Prognosis of 13 Cases of Acute Erythroleukemia

The aim of this study was to investigate the clinical characteristics and prognosis of acute erythroleukemia （AEL, AML-M6 ）. The clinical features and results of morphologic, immunophenotypic, cytogenetic and molecular biologic detections were retrospectively analyzed in 13 cases of AEL from 305 acute leukemia patients hospitalized between october 2007 and october 2012. The results showed that the expression of erythroid and non-erythroid cells increased at the same time. The myeloid antigens mainly expressed CD13/CD33/CDllT/CD34, while the erythroid antigens expressed Gly and CD71. The karyotypic detection indicated that there were 1 case with normal karyotype, 3 cases with simple karyotypic abnormalitoy and 2 cases with complex karyotypic abnormality, the other cases were not detected. The molecular biological detection found that the poor prognosis gene existed in 5 cases 138.5% （5/13）1 , including 3 cases with MLL-MLL fusion gene, 1 case with MLL mutation, and 1 cases with NRAS gene mutation, the abnormal genes were not detected in remainder 8 cases. After chemotherapy with decitabine, the complete remission （CR） rate achieved 53.5 % （ 7/13 ） , partial remission （ DR ） rate achieved 15.4 % （ 2/13 ）. Finally, 8 patients received allo-HSCT, the median overall survival （ OS ） was 20.7 months, 3 year survival rate was 79%, 3 year disease-free survival rate was 78%. It is concluded that the acute erythroleukemia is a rare subtype of AML, which is transformed from MDS and has harmful genes and poor prognosis. Allo-HSCT and treatment with decitabine may enhance the survival rate of AEL.