Divisions of Hematology, Mayo Clinic, Rochester, MN, USA.
Blood. 2011 Oct 27;118(17):4595-8. doi: 10.1182/blood-2011-08-371096. Epub 2011 Aug 31.
DIPSS-plus (the Dynamic International Prognostic Scoring System-plus) includes 8 risk factors for survival in primary myelofibrosis. In the present study of 884 karyotypically annotated patients with primary myelofibrosis, we sought to identify 1 or 2 parameters that can reliably predict death in the first 2 years of disease. After a median of 8.2 years from time of referral to the Mayo Clinic, 564 deaths (64% of patients in the study) had been recorded. Risk factors associated with > 80% 2-year mortality included monosomal karyotype, inv(3)/i(17q) abnormalities, or any 2 of the following: circulating blasts > 9%, leukocytes ≥ 40 × 10(9)/L, or other unfavorable karyotype. Patients with any 1 of these risk profiles (n = 52) displayed significantly shorter overall survival than those otherwise belonging to a high-risk category per DIPSS-plus (n = 298); respective median survivals were 9 and 23 months (hazard ratio 2.2, 95% confidence interval 1.6-3.1; P < .01). The present information complements DIPSS-plus in the selection of primary myelofibrosis patients for high-risk treatment approaches.
DIPSS-plus（动态国际预后评分系统-plus）包括原发性骨髓纤维化中 8 个与生存相关的风险因素。在本研究中，对 884 例核型注释的原发性骨髓纤维化患者进行了研究，我们试图确定 1 或 2 个参数，以可靠地预测疾病的前 2 年的死亡风险。从转诊到梅奥诊所开始，中位时间为 8.2 年后，记录了 564 例死亡（研究中患者的 64%）。与 2 年死亡率超过 80%相关的危险因素包括单体核型、inv(3)/i(17q)异常，或以下任何两种情况：循环原始细胞 > 9%、白细胞≥40×10(9)/L，或其他不良核型。具有这些风险特征之一的患者（n=52）与根据 DIPSS-plus 否则属于高危类别（n=298）的患者相比，总体生存率显著缩短；分别为 9 个月和 23 个月（风险比 2.2，95%置信区间 1.6-3.1；P<0.01）。目前的信息补充了 DIPSS-plus，有助于选择原发性骨髓纤维化患者进行高危治疗方法。
