CAJ | 학술논문

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FAB-M2,5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HLA-DR,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)시급성수성백혈병최상견적염색체이상지일.t(8;21)AML구유독특적림상특정,화료완해솔교고,생존기교장,단역유예후교차적보도.위진일보탐토중국인군t(8;21)AML적림상특정급예후인소,대75례t(8;21)AML환자,기중포괄68례FAB-M2,5례M4,2례M5진행료회고성분석.결과표명:39례환자골수중가견Auer소체(52%),이골수기산세포증다(＞5%)견우5례(6.7%);면역분형고표체CD34화HLA-DR,부유13례환자표체CD19(20.9%);세포유전학분석시62.5%환자구유부가염색체이상,주요부가이상류형위성염색체주실(LOS),+4,del(9q)급+8;통과상규유도화료,완전완해솔82.7%;수방1-96월,19례복발,중위복발시간위10.5월(3-42월),중위생존시간시20개월,5년예기생존솔위32.3%;다인소분석현시염색체핵형,수외백혈병,년령급완해후치료방식시영향생존적주요예후인소;반유부가염색체이상환자적생존기교단순t(8;21)환자단(P=0.019),이불동부가이상지간무명현차이;수외백혈병역시불량적예후인소(P=0.012),년령≤15세환자비년령＞15세자생존기장(P=0.045);접수조혈간세포이식적환자적예후호우단순화료자(P=0.030).결론:중국인군t(8;21)AML구유불동우기타인군적특성,기예후상대교차,우기시반유부가염색체이상급수외백혈병자예후경차;대우구유불량예후인소적t(8;21)AML환자응해건의접수조혈간세포이식.
The translocation t(8;21) (q22;q22) frequently associated with additional chromosomal aberrations is one of the most recurrent chromosomal abnormalities in AML. Clinically, this type of AML usually shows some specific characteristics and has a good response to chemotherapy with a high remission rate and a relatively long median survival.On the other hand, some reports also showed poor prognosis in AML patients with t(8;21 ) , and the associated bad prognosis factors have not been strongly established to date. To investigate this issue and to further identify the related characteristics of t(8 ;21 ) AML in China, 75 Chinese AML patients with t(8 ;21 ) were retrospectively analyzed. They comprised 68 cases of M2, five of M4 and two of M5 according to FAB classification. The results indicated that Auer rods were observed in 39 patients (52%) and marrow eosinophilia was detected in only 5 patients (6.7%). These patients showed high level of HLA-DR and CD34 expression, while CD19 was detected in only 13 patients (20.9%). Cytogenetically, 62.5 % cases had additional chromosomal abnormalities, and the main associated recurrent additional abnormalities were loss of a sex chromosome (LOS), trisomy 4, del (9q) and trisomy 8. After conventional induction therapy, 62 patients attained complete remission (CR) resulting in the CR rate 82.7%. With a follow-up of 1 to 96 months, 19 cases relapsed at a median time of 10.5 months (range 3 to 42 months). The median overall survival was 20 months, and the estimated 5-year overall survival (OS) rate was 32.3%. In multivariate analyses of prognostic factors,karyotype, extramedullary leukemia, age and post-remission therapy were of prognostic value for OS. Patients with additional chromosomal anomalies had shorter survival compared to those with t(8 ;21 ) only (P = 0.019), no matter which kind of additional karyotype it was. Extramedullary leukemia was an adverse prognostic factor ( P = 0. 012). Patients aged 15 years or less had a longer survival than those aged more than 15 years (P =0.045). Patients accepted HSCT in post-remission therapy had better outcome compared to those with chemotherapy only. It is concluded that Chinese AML patients with t(8 ;21 ) had some different characteristics as compared with patients from other countries, a relatively poor outcome was observed in our patients, especially in those with extramedullary leukemia or additional chromosomal abnormalities. HSCT should be recommended to t(8 ;21 ) AML in China, especially to those with adverse prognostic factors.