Li Y, Liu Q, Wang Z, Qin Y Z, Dang H, Shi Y, He Q, Jiang Q, Jiang H, Lai Y Y
Peking University People's Hospital, Peking University Institute of Hematology, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Beijing 100044, China.
Zhonghua Xue Ye Xue Za Zhi. 2019 Mar 14;40(3):195-199. doi: 10.3760/cma.j.issn.0253-2727.2019.03.006.
To analyze the characteristics of myeloid neoplasms with t (3;21) (q26;q22) . Clinical data of patients with t (3; 21) (q26; q22) , diagnosed as hematologic malignancies in Peking University people's hospital from January 2011 to March 2018, were collected retrospectively. 19 patients in our hospital and forty-eight patients bearing t (3;21) (q26;q22) with detailed survival data reported in literature were summarized. Kaplan- Meier method was used for survival analysis. Among 19 patients, including 15 males and 4 females with a median age of 36 years (22-68 years) , 4 cases was diagnosed as de novo acute myeloid leukemia (AML) , 4 as myelodysplastic syndromes (MDS) , 3 as MDS-AML and 8 as chronic myelogenous leukemia (CML) in myeloid blast transformation. All of the 19 patients were detected to have t (3;21) (q26;q22) by G-banding technique and 13 carried additional cytogenetic aberrations. 9 of the 19 patients were detected for positive AML1-MDS1 fusion genes. In the 9 patients with detailed follow-up data, 6 patients received chemotherapy and only 2 achieved complete remission (CR) while 4 with no response. During the follow-up period, 8 patients died and the median overall survival (OS) was 6 months (4.5 to 22 months) . Survival analysis of the present 9 patients together with the literature data showed that the prognosis was poor and the median OS was 7 months. In particular, AML/t-AML had the worst prognosis. Hematopoietic stem cell transplantation (HSCT) could significantly improve survival, the median OS in HSCT group and non-HSCT group were 20.9 and 4.7 months respectively (<0.001) . t (3; 21) (q26; q22) is a rare recurrent chromosomal abnormality which is detected mainly in myeloid neoplasm and confer to poor clinical prognosis. HSCT should be recommended to improve the outcomes.
分析伴t(3;21)(q26;q22)的髓系肿瘤的特征。回顾性收集2011年1月至2018年3月在北京大学人民医院被诊断为血液系统恶性肿瘤的伴t(3;21)(q26;q22)患者的临床资料。总结了我院的19例患者以及文献报道的48例有详细生存数据的伴t(3;21)(q26;q22)患者。采用Kaplan-Meier法进行生存分析。19例患者中，男性15例，女性4例，中位年龄36岁（22 - 68岁），4例诊断为初发急性髓系白血病（AML），4例为骨髓增生异常综合征（MDS），3例为MDS-AML，8例为慢性髓性白血病（CML）髓系原始细胞转化。19例患者均通过G显带技术检测到t(3;21)(q26;q22)，13例伴有其他细胞遗传学异常。19例患者中有9例检测到AML1-MDS1融合基因阳性。在9例有详细随访数据的患者中，6例接受了化疗，仅2例达到完全缓解（CR），4例无反应。随访期间，8例患者死亡，中位总生存期（OS）为6个月（4.5至22个月）。对这9例患者与文献数据进行生存分析显示预后较差，中位OS为7个月。特别是，AML/t-AML预后最差。造血干细胞移植（HSCT）可显著提高生存率，HSCT组和非HSCT组的中位OS分别为20.9个月和4.7个月（<0.001）。t(3;21)(q26;q22)是一种罕见的复发性染色体异常，主要在髓系肿瘤中检测到，且临床预后较差。应推荐HSCT以改善预后。
