Cui Ningbo, Fu Rong, Qu Wen, Ruan Erbao, Wang Xiaoming, Wang Guojin, Wu Yuhong, Liu Hong, Guan Jing, Song Jia, Xing Limin, Li Lijuan, Jiang Huijuan, Liu Hui, Wang Yihao, Liu Chunyan, Zhang Wei, Wang Huaquan, Shao Zonghong
Department of Hematology, General Hospital, Tianjin Medical University, Tianjin 300052, China.
Department of Hematology, General Hospital, Tianjin Medical University, Tianjin 300052, China; Email:
Zhonghua Yi Xue Za Zhi. 2015 Aug 25;95(32):2620-2.
To investigate the clinical characteristics and risk factors of monosomy 7 malignant clonal evolution in patients with severe aplastic anemia (SAA) treated with combined immunosuppressive therapy (IST).
The clinical data of SAA patients treated with IST who had monosomy 7 malignant clonal evolution from October 2004 to January 2012 were analyzed respectively.
Six patients (4.2%) had monosomy 7 clonal evolutions. The median time to monosomy 7 was 36 (12-75) months after IST. All 6 patients were diagnosed myelodysplastic syndromes (MDS). Among them, 3 patients transformed to acute myeloid leukemia following MDS. The time was 24, 45 and 51 months after IST. The median following time was 42 (17-84) months. Four patients died during the following time. The median time from MDS to death was 9 (5-17) months. Among them, three patients died with infection, one died with cerebral hemorrhage. Six patients had the clinical characteristics that they had no response to IST after 6 months, high monocyte percentage in one month after IST combined with recombinant human granulocyte colony stimulating factor (rHu-GCSF) and agranulocytosis in 3 months after IST.
Poor myeloid response to IST suggests malignant clonal hematopoiesis and poor prognosis in SAA patients.
探讨接受联合免疫抑制治疗（IST）的重型再生障碍性贫血（SAA）患者7号染色体单体恶性克隆演变的临床特征及危险因素。
对2004年10月至2012年1月期间接受IST治疗且发生7号染色体单体恶性克隆演变的SAA患者的临床资料进行分析。
6例患者（4.2%）发生了7号染色体单体克隆演变。IST后出现7号染色体单体的中位时间为36（12 - 75）个月。所有6例患者均被诊断为骨髓增生异常综合征（MDS）。其中，3例患者在MDS后转化为急性髓系白血病。时间为IST后24、45和51个月。中位随访时间为42（17 - 84）个月。4例患者在随访期间死亡。从MDS到死亡的中位时间为9（5 - 17）个月。其中，3例死于感染，1例死于脑出血。6例患者具有以下临床特征：IST治疗6个月后无反应、IST联合重组人粒细胞集落刺激因子（rHu - GCSF）治疗1个月后单核细胞百分比高以及IST治疗3个月后粒细胞缺乏。
IST治疗后髓系反应不佳提示SAA患者存在恶性克隆造血且预后不良。
