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t(8;21)是急性髓性白血病最常见的染色体异常之一。t(8;21)AML具有独特的临床特征,化疗缓解率较高,生存期较长,但亦有预后较差的报道。为进一步探讨中国人群t(8;21)AML的临床特征及预后因素,对75例t(8;21)AML患者,其中包括68例FABM2,5例M4,2例M5进行了回顾性分析。结果表明:39例患者骨髓中可见Auer小体(52%),而骨髓嗜酸细胞增多(>5%)见于5例(6.7%);免疫分型高表达CD34和HLADR,仅有13例患者表达CD19(20.9%);细胞遗传学分析示62.5%患者具有附加染色体异常,主要附加异常类型为性染色体丢失(LOS),+4,del(9q)及+8;通过常规诱导化疗,完全缓解率82.7%;随访1-96月,19例复发,中位复发时间为10.5月(3-42月),中位生存时间是20个月,5年预期生存率为32.3%;多因素分析显示染色体核型,髓外白血病,年龄及缓解后治疗方式是影响生存的主要预后因素;伴有附加染色体异常患者的生存期较单纯t(8;21)患者短(P=0.019),而不同附加异常之间无明显差异;髓外白血病亦是不良的预后因素(P=0.012),年龄≤15岁患者比年龄>15岁者生存期长(P=0.045);接受造血干细胞移植的患者的预后好于单纯化疗者(P=0.030)。结论:中国人群t(8;21)AML具有不同于其他人群的特性,其预后相对较差,尤其是伴有附加染色体异常及髓外白血病者预后更差;对于具有不良预后因素的t(8;21)AML患者应该建议接受造血干细胞移植。
t (8; 21) is one of the most common chromosomal abnormalities in acute myeloid leukemia. t (8; 21) AML has a unique clinical features, high response rate of chemotherapy, longer survival, but there are also reports of poor prognosis. To further investigate the clinical features and prognostic factors of t (8; 21) AML in Chinese population, 75 patients with t (8; 21) AML, including 68 FABM2, 5 patients with M4 and 2 patients with M5, were retrospectively analyzed. The results showed that Auer bodies (52%) were seen in the bone marrow of 39 patients, and 5 (6.7%) were found in the bone marrow with eosinophilia (> 5%). CD34 and HLADR were highly expressed in the immunophenotype and only 13 patients (20.9%). Cytogenetic analysis revealed 62.5% of patients had additional chromosomal abnormalities with major additional abnormalities of sex chromosome loss (LOS), +4, del (9q) and +8; complete remission by conventional induction chemotherapy Rate of 82.7%; Follow-up 1-96 months, 19 cases of recurrence, the median recurrence time was 10.5 months (3-42 months), the median survival time was 20 months, 5-year expected survival rate was 32.3%; multivariate analysis showed Chromosome karyotype, extramedullary leukemia, age, and post-remission treatment were the main prognostic factors for survival. Patients with additional chromosomal abnormalities had shorter survival (t = 8), patients with t No significant difference between the abnormalities; extramedullary leukemia is also a poor prognostic factor (P = 0.012), patients aged ≤ 15 years longer than those aged> 15 years (P = 0.045); prognosis of patients receiving hematopoietic stem cell transplantation Better than chemotherapy alone (P = 0.030). CONCLUSION: The Chinese population of t (8; 21) AML has characteristics different from those of other populations, and its prognosis is relatively poor, especially in patients with additional chromosomal abnormalities and extramedullary leukemia. The prognosis of patients with untreated prognosis is t (8 ; 21) AML patients should be recommended for hematopoietic stem cell transplantation.