孤立11q缺失骨髓增生异常综合征患者的特征和生存结局

IF 2.1 4区 医学 Q3 HEMATOLOGY
Kensuke Takaoka , Rami Komrokji , Kelly Chien , Guillermo Montalban-Bravo , Julie Braish Salman , Samuel Urrutia , Alex Bataller , Alexandre Bazinet , Jeannot Kekedjian , Najla H. Al Ali , David Sallman , Eric Padron , Zhuoer Xie , Rashmi Kanagal-Shamanna , Guilin Tang , Hui Yang , Juan Jose Rodriguez-Sevilla , Guillermo Garcia-Manero , Koji Sasaki
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引用次数: 0

摘要

骨髓增生异常综合征(MDS)伴分离性11q缺失是一种罕见的有利细胞遗传学异常,其进展为急性髓性白血病(AML)的风险较低。本研究的目的是描述孤立性del(11q) MDS患者的临床特征和长期预后。1997年8月至2024年1月期间,52例MDS和孤立性del(11q)患者被诊断出来,占队列的0.4% %。中位年龄为69岁,男性轻度占优势(62% %)。根据世界卫生组织(WHO) 2022年的数据,42% %的MDS患者伴有低原爆。中位随访96个月,中位生存期为71个月,5年生存率为53. %。低甲基化药物组和最佳支持治疗组的5年生存率分别为45 %和68 % (P = 0.63)。多变量Cox回归分析确定年龄、绝对中性粒细胞计数、血红蛋白和原细胞百分比是重要的预后因素。尽管孤立的del(11q) MDS被归类为极好风险的细胞遗传学异常,但长期生存率很低,伴有进展为AML和细胞减少并发症的风险。较差的长期生存率表明需要研究有效的支持性护理和早期干预,以使具有低风险MDS和高风险特征的患者受益。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Characteristics and survival outcomes of patients with myelodysplastic syndromes with isolated 11q deletion
Myelodysplastic syndrome (MDS) with isolated deletion 11q is a rare favorable cytogenetic abnormality with a low risk of progression to acute myeloid leukemia (AML). The aim of this study is to describe the clinical characteristics and long-term outcomes of patients with isolated del(11q) MDS. Between August 1997 and January 2024, 52 patients with MDS and isolated del(11q) were diagnosed, representing 0.4 % of the cohort. The median age was 69 years, with a mild male predominance (62 %). By the World Health Organization (WHO) 2022, 42 % of patients had MDS with low blasts. With a median follow-up of 96 months, the median survival was 71 months with a 5-year survival rate of 53 %. The 5-year survival rates were 45 % and 68 % in the hypomethylating agents and best supportive care group, respectively (P = 0.63). Multivariate Cox regression analyses identified age, absolute neutrophil count, hemoglobin, and blast percentages as significant prognostic factors. Despite isolated del(11q) MDS being classified as a very-good-risk cytogenetic abnormality, long-term survival is poor with the risk of progression to AML and complications from cytopenias. The poor long-term survival indicates the need for the investigation of effective supportive care and early intervention to benefit patients with lower-risk MDS and high-risk features.
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来源期刊
Leukemia research
Leukemia research 医学-血液学
CiteScore
4.00
自引率
3.70%
发文量
259
审稿时长
1 months
期刊介绍: Leukemia Research an international journal which brings comprehensive and current information to all health care professionals involved in basic and applied clinical research in hematological malignancies. The editors encourage the submission of articles relevant to hematological malignancies. The Journal scope includes reporting studies of cellular and molecular biology, genetics, immunology, epidemiology, clinical evaluation, and therapy of these diseases.
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