Solé F, Espinet B, Sanz G F, Cervera J, Calasanz M J, Luño E, Prieto F, Granada I, Hernández J M, Cigudosa J C, Diez J L, Bureo E, Marqués M L, Arranz E, Ríos R, Martínez Climent J A, Vallespí T, Florensa L, Woessner S
Hospital de l'Esperança/Hospital del Mar/Hospital Central l'Aliança (Barcelona), Hospital la Fé (Valencia), Spain.
Br J Haematol. 2000 Feb;108(2):346-56. doi: 10.1046/j.1365-2141.2000.01868.x.
Recently, a consensus International Prognostic Scoring System (IPSS) for predicting outcome and planning therapy in the myelodysplastic syndromes (MDS) has been developed. However, the intermediate-risk cytogenetic subgroup defined by the IPSS includes a miscellaneous number of different single abnormalities for which real prognosis at present is uncertain. The main aims of this study were to evaluate in an independent series the prognostic value of the IPSS and to identify chromosomal abnormalities with a previously unrecognized good or poor prognosis in 640 patients. In univariate analyses, cases with single 1q abnormalities experienced poor survival, whereas those with trisomy 8 had a higher risk of acute leukaemic transformation than the remaining patients (P = 0.004 and P = 0.009 respectively). Patients with single del(12p) had a similar survival to patients with a normal karyotype and showed some trend for a better survival than other cases belonging to the IPSS intermediate-risk cytogenetic subgroup (P = 0.045). Multivariate analyses demonstrated that IPSS cytogenetic prognostic subgroup, proportion of bone marrow blasts and haemoglobin level were the main prognostic factors for survival, and the first two characteristics and platelet count were the best predictors of acute leukaemic transformation risk. A large international co-operative study should be carried out to clarify these findings.
最近，已开发出一种用于预测骨髓增生异常综合征（MDS）预后及规划治疗的国际预后评分系统（IPSS）共识。然而，IPSS定义的中危细胞遗传学亚组包含多种不同的单一异常，目前其真实预后尚不确定。本研究的主要目的是在一个独立队列中评估IPSS的预后价值，并在640例患者中识别出预后良好或不良但此前未被认识到的染色体异常。在单因素分析中，伴有单一1q异常的病例生存较差，而伴有8号染色体三体的病例急性白血病转化风险高于其余患者（分别为P = 0.004和P = 0.009）。伴有单一del(12p)的患者与核型正常的患者生存相似，且与属于IPSS中危细胞遗传学亚组的其他病例相比，有生存更好的趋势（P = 0.045）。多因素分析表明，IPSS细胞遗传学预后亚组、骨髓原始细胞比例和血红蛋白水平是生存的主要预后因素，前两个特征和血小板计数是急性白血病转化风险的最佳预测指标。应开展一项大型国际合作研究以阐明这些发现。
