Lucijanic Marko, Veletic Ivo, Rahelic Dario, Pejsa Vlatko, Cicic David, Skelin Marko, Livun Ana, Tupek Katarina Marija, Stoos-Veic Tajana, Lucijanic Tomo, Maglicic Ana, Kusec Rajko
Hematology Department, University Hospital Dubrava, Av. Gojka Suska 6, 10000, Zagreb, Croatia.
Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Wien Klin Wochenschr. 2018 Feb;130(3-4):126-133. doi: 10.1007/s00508-018-1318-z. Epub 2018 Jan 25.
Primary and secondary myelofibrosis (PMF and SMF) are malignant diseases of hematopoietic stem cell characterized by the neoplastic myeloproliferation and a strong inflammatory milieu. The prognostic nutritional index (PNI) integrates information on albumin and absolute lymphocyte count (ALC) and reflects the inflammatory, nutritional and immune status of a patient. The clinical and prognostic significance of albumin, ALC and PNI in patients with myelofibrosis has not been previously investigated.
We retrospectively analyzed a cohort of 83 myelofibrosis patients treated in our institution from 2006 to 2017. Albumin, ALC and PNI were assessed in addition to other disease specific markers.
The PMF and SMF patients had significantly lower ALC and PNI but similar albumin compared to controls. Lower albumin was significantly associated with older age and parameters reflecting more aggressive disease biology (e.g. anemia, lower platelet levels, higher lactate dehydrogenase (LDH), circulatory blasts, transfusion dependency, blast phase disease), inflammation (higher C reactive protein (CRP), constitutional symptoms) and higher degree of bone marrow fibrosis. Lower ALC was significantly associated with lower white blood cells (WBC) and lower circulatory blasts. Low PNI was associated with lower albumin, lower ALC, anemia, lower WBCs, lower serum iron and lower transferrin saturation. There was no difference in albumin, ALC and PNI regarding the driver mutations. In multivariate analysis adjusted for age and gender, low albumin (hazard ratio [HR] = 4.61, P = 0.001), low ALC (HR = 3.54, P = 0.004) and Dynamic International Prognostic Scoring System (DIPSS) (HR = 2.45, P = 0.001) were able to predict inferior survival independently of each other. Accordingly, low PNI (HR = 4.32, P < 0.001) predicted poor survival independently of DIPSS (HR = 3.31, P < 0.001).
Assessing albumin, ALC and PNI might improve prognostication in patients with myelofibrosis and could assist in recognition of patients under increased risk of death.
原发性和继发性骨髓纤维化(PMF和SMF)是造血干细胞的恶性疾病,其特征为肿瘤性骨髓增殖和强烈的炎症环境。预后营养指数(PNI)整合了白蛋白和绝对淋巴细胞计数(ALC)的信息,反映了患者的炎症、营养和免疫状态。此前尚未研究白蛋白、ALC和PNI在骨髓纤维化患者中的临床及预后意义。
我们回顾性分析了2006年至2017年在我院接受治疗的83例骨髓纤维化患者的队列。除了其他疾病特异性标志物外,还评估了白蛋白、ALC和PNI。
与对照组相比,PMF和SMF患者的ALC和PNI显著降低,但白蛋白水平相似。较低的白蛋白与年龄较大以及反映疾病生物学行为更具侵袭性的参数(如贫血、较低的血小板水平、较高的乳酸脱氢酶(LDH)、循环原始细胞、输血依赖、原始细胞期疾病)、炎症(较高的C反应蛋白(CRP)、全身症状)和较高程度的骨髓纤维化显著相关。较低的ALC与较低的白细胞(WBC)和较低的循环原始细胞显著相关。低PNI与较低的白蛋白、较低的ALC、贫血、较低的WBC、较低的血清铁和较低的转铁蛋白饱和度相关。关于驱动基因突变,白蛋白、ALC和PNI没有差异。在根据年龄和性别进行调整的多变量分析中,低白蛋白(风险比[HR]=4.61,P=0.001)、低ALC(HR=3.54,P=0.004)和动态国际预后评分系统(DIPSS)(HR=2.45,P=0.001)能够相互独立地预测较差的生存率。因此,低PNI(HR=4.32,P<0.001)独立于DIPSS(HR=3.31,P<0.001)预测生存不良。
评估白蛋白、ALC和PNI可能会改善骨髓纤维化患者的预后,并有助于识别死亡风险增加的患者。