Department of Hematology, Vall d'Hebrón University Hospital, Pg. Vall d'Hebrón 119-129, 08035-Barcelona, Spain.
J Clin Oncol. 2013 Mar 1;31(7):916-22. doi: 10.1200/JCO.2012.41.6073. Epub 2013 Jan 14.
Complex karyotype (CK) is the poorest risk factor in patients with myelodysplastic syndrome (MDS). It has recently been reported that monosomal karyotype (MK) worsens the prognosis of patients with CK. PATIENTS AND METHODS; We analyzed 1,054 adult patients with MDS with an abnormal karyotype from the Spanish Registry of MDS. The aim of the study was to describe the incidence, characteristics, and prognosis of MK; the main end points were overall survival (OS) and leukemia-free survival.
MK was identified in 172 patients (16%), most of whom (88%) presented with CK. Variables significantly associated with OS were age (hazard ratio [HR], 1.90; P < .001), bone marrow (BM) blast percentage (HR, 1.05; P < .001), hemoglobin level (HR, 1.71; P < .001), platelet count (HR, 1.41; P < .001), karyotype complexity (CK [three abnormalities]: HR, 1.81; P = .003; very CK [> three abnormalities]: HR, 2; P < .001), and abnormalities of chromosome 5 and/or 7 (HR, 1.89; P < .001). Variables significantly related to the risk of transformation to acute myeloid leukemia (AML) were higher BM blast percentage (HR, 1.12; P < .001) and karyotype complexity (CK: HR, 2.53; P = .002; very CK: HR, 2.77; P < .001).
After accounting for karyotype complexity, MK was not associated with OS or evolution to AML. In conclusion, these results demonstrate that the prognostic value of MK in MDS is not independent and is mainly the result of its strong association with number of chromosomal abnormalities.
复杂核型(CK)是骨髓增生异常综合征(MDS)患者最差的风险因素。最近有报道称,单体核型(MK)会恶化 CK 患者的预后。
我们分析了来自西班牙 MDS 注册中心的 1054 例异常核型的成人 MDS 患者。该研究的目的是描述 MK 的发生率、特征和预后;主要终点是总生存期(OS)和无白血病生存期。
在 172 例患者(16%)中发现了 MK,其中大多数(88%)患者伴有 CK。与 OS 显著相关的变量为年龄(风险比 [HR],1.90;P <.001)、骨髓(BM)原始细胞百分比(HR,1.05;P <.001)、血红蛋白水平(HR,1.71;P <.001)、血小板计数(HR,1.41;P <.001)、核型复杂性(CK [三种异常]:HR,1.81;P =.003;非常 CK [> 三种异常]:HR,2;P <.001)和 5 号和/或 7 号染色体异常(HR,1.89;P <.001)。与向急性髓系白血病(AML)转化的风险显著相关的变量为 BM 原始细胞百分比较高(HR,1.12;P <.001)和核型复杂性(CK:HR,2.53;P =.002;非常 CK:HR,2.77;P <.001)。
在考虑核型复杂性后,MK 与 OS 或向 AML 进展无关。总之,这些结果表明,MK 在 MDS 中的预后价值不是独立的,主要是由于其与染色体异常数量的强烈关联。