Lu Lin, Xiao Zhi-jian, Chen Hui-shu, Lin Hua, Han Zhong-chao
Institute of Hematology and Blood Disease Hospital, CAMS & PUMC, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2005 May;26(5):281-4.
To investigate the clinical and hematological features and identify the prognostic factors associated with short-term survival in primary myelofibrosis (PMF) patients under 45 years old.
The clinical manifestations, laboratory parameters, and survival were retrospectively analyzed in 56 PMF patients under 45 years old. Univariate analysis of prognostic factors was performed using Logrank test and multivariate analysis using COX model.
Of the 56 patients, 27 were males and 29 females. The range of age was from 20 to 45 years (median 38 years). 84% of the patients were anemic and 66% Hb < 100 g/L. 32% of the patients had constitutional symptoms including fever, night sweats and weight loss. The median survival was 69 months (95% CI 11-127). By univariate analysis, Hb < 100 g/L, platelet count < 100 x 10(9)/L, WBC < 10 x 10(9)/L, constitutional symptoms and the duration from first signs to diagnosis < or = 6 months were associated with poor prognosis. By multivariate analysis, only Hb < 100 g/L and constitutional symptoms were independently associated with short survival. With these two adverse prognostic factors, the patients could be separated into a high risk and a low risk groups, and the median survivals were 16 and 88 months, respectively (P < 0.001). Using these two factors to predict the less than 3-years survival for individual patient, the sensitivity, specificity and positive predictive value were 67%, 100% and 100%, respectively.
Hb < 100 g/L and constitutional symptoms in PMF patients under 45 years old were significantly associated with short survival and poor prognosis. These two prognostic factors enabled to separate patients into a high and a low risk groups. The survival of high-risk patients was less than three years.
探讨45岁以下原发性骨髓纤维化（PMF）患者的临床和血液学特征，并确定与短期生存相关的预后因素。
对56例45岁以下的PMF患者的临床表现、实验室参数和生存情况进行回顾性分析。采用Logrank检验进行预后因素的单因素分析，采用COX模型进行多因素分析。
56例患者中，男性27例，女性29例。年龄范围为20至45岁（中位年龄38岁）。84%的患者贫血，66%血红蛋白<100g/L。32%的患者有全身症状，包括发热、盗汗和体重减轻。中位生存期为69个月（95%CI 11-127）。单因素分析显示，血红蛋白<100g/L、血小板计数<100×10⁹/L、白细胞计数<10×10⁹/L、全身症状以及从首发症状到诊断的时间≤6个月与预后不良相关。多因素分析显示，只有血红蛋白<100g/L和全身症状与短期生存独立相关。根据这两个不良预后因素，患者可分为高危组和低危组，中位生存期分别为16个月和88个月（P<0.001）。用这两个因素预测个体患者3年以内的生存情况，敏感性、特异性和阳性预测值分别为67%、100%和100%。
45岁以下PMF患者血红蛋白<100g/L和全身症状与短期生存及不良预后显著相关。这两个预后因素可将患者分为高危组和低危组。高危患者的生存期不足3年。
