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目的研究45岁以下原发性骨髓纤维化(PMF)患者的临床特点和影响生存期的预后因素。方法分析56例年龄≤45岁的PMF患者的临床特征和实验室参数,确定影响生存期的预后因素。单因素分析用Logrank检验,多因素分析用COX回归模型。结果56例患者中,男27例,女29例,84%患者有贫血,66%患者Hb<100g/L,32%患者有低热、盗汗和消瘦等全身症状。中位生存期为69(11～127)个月。单因素分析显示外周血Hb<100g/L、BPC<100×109/L、WBC<10×109/L、有全身症状及从发病到诊断间隔时间≤6个月是影响患者生存期的危险因素。多因素分析显示Hb<100g/L和有全身症状与生存期短显著相关。以是否同时具有2项不良指标为标准将患者分为高危组和低危组。高危组患者生存期(16个月)明显短于低危组患者(88个月)(P<0.01)。用此2项指标预测生存期<3年的敏感性、特异性和阳性预测值分别为67%、100%和100%。结论对45岁以下PMF患者,Hb<100g/L和有全身症状是生存期短的预后不良因素,用此2项指标可将患者分为高危组和低危组,高危组患者生存期短于3年。
Objective To study the clinical characteristics and prognostic factors of survival in patients with primary myelofibrosis (PMF) under 45 years of age. Methods The clinical features and laboratory parameters of 56 PMF patients aged ≤ 45 years were analyzed to determine the prognostic factors affecting survival. Univariate analysis using Logrank test, multivariate analysis with COX regression model. Results Of the 56 patients, 27 were male and 29 were female, 84% had anemia, 66% had Hb <100g / L, and 32% had generalized symptoms of fever, night sweats and weight loss. The median survival was 69 (11-127) months. Univariate analysis showed that peripheral blood Hb <100g / L, BPC <100 × 109 / L, WBC <10 × 109 / L, with systemic symptoms and the interval from onset to diagnosis ≤ 6 months were the risk factors affecting the survival of patients . Multivariate analysis showed that Hb <100g / L and systemic symptoms were significantly associated with short survival. The patients were divided into high-risk group and low-risk group according to whether they had two adverse indicators at the same time. Patients in the high-risk group had a significantly shorter survival (16 months) than those in the low-risk group (88 months) (P <0.01). The sensitivity, specificity, and positive predictive value for survival of <3 years using these two measures were 67%, 100%, and 100%, respectively. Conclusions For patients with PMF below 45 years of age, Hb <100g / L and systemic symptoms are poor prognostic factors of short survival. Using these two indicators, patients can be divided into high-risk group and low-risk group. Patients in high-risk group have shorter survival time 3 years.