Lucijanic Marko, Livun Ana, Stoos-Veic Tajana, Pejsa Vlatko, Jaksic Ozren, Cicic David, Lucijanic Jelena, Romic Zeljko, Orehovec Biserka, Aralica Gorana, Miletic Marko, Kusec Rajko
a Hematology Department , University Hospital Dubrava , Zagreb , Croatia.
b Divison of Molecular Diagnosis and Genetics, Clinical Department of Laboratory Diagnostics , University Hospital Dubrava , Zagreb , Croatia.
Hematology. 2018 May;23(4):201-207. doi: 10.1080/10245332.2017.1376843. Epub 2017 Sep 14.
To investigate the clinical and prognostic significance of absolute basophil count (ABC) in patients with primary myelofibrosis (PMF).
We retrospectively investigated 58 patients with PMF treated in our institution in the period from 2006 to 2017. ABC was obtained in addition to other hematological and clinical parameters. Patients were separated into high and low ABC groups using the Receiver operating characteristic curve analysis.
ABC was higher in PMF patients than in healthy controls (P < 0.001). Patients with high ABC had higher white blood cells (P < 0.001), higher red cell distribution width (P = 0.035), higher lactate dehydrogenase (P < 0.001), more frequently had circulatory blasts (P < 0.001), constitutional symptoms (P = 0.030) and massive splenomegaly (P = 0.014). ABC was also positively correlated with absolute monocyte count (AMC) (P < 0.001) and other components of differential blood count. There was no difference in ABC regarding driver mutations or degree of bone marrow fibrosis. Univariately, high ABC was significantly associated with inferior overall survival (hazard ratio (HR) 4.79, P < 0.001). This effect remained statistically significant (HR 4.27, P = 0.009) in a multivariate Cox regression model adjusted for age, gender, Dynamic International Prognostic Scoring System (HR 2.6, P = 0.001) and AMC (HR 8.45, P = 0.002).
High ABC reflects higher disease activity and stronger proliferative potential of disease. ABC and AMC independently predict survival and therefore seem to reflect different underlying pathophysiologic processes. Hence, both have a potential for improvement of current prognostic scores.
Basophils represent a part of malignant clone in PMF and are associated with unfavorable disease features and poor prognosis which is independent of currently established prognostic scoring system and monocytosis.
探讨原发性骨髓纤维化（PMF）患者绝对嗜碱性粒细胞计数（ABC）的临床及预后意义。
我们回顾性研究了2006年至2017年期间在我院接受治疗的58例PMF患者。除其他血液学和临床参数外，还获取了ABC。使用受试者工作特征曲线分析将患者分为ABC高组和低组。
PMF患者的ABC高于健康对照组（P＜0.001）。ABC高的患者白细胞更高（P＜0.001）、红细胞分布宽度更高（P = 0.035）、乳酸脱氢酶更高（P＜0.001），循环原始细胞更常见（P＜0.001）、有全身症状（P = 0.030）和巨脾（P = 0.014）。ABC还与绝对单核细胞计数（AMC）（P＜0.001）及其他血常规成分呈正相关。ABC在驱动基因突变或骨髓纤维化程度方面无差异。单因素分析显示，高ABC与较差的总生存期显著相关（风险比[HR] 4.79，P＜0.001）。在调整了年龄、性别、动态国际预后评分系统（HR 2.6，P = 0.001）和AMC（HR 8.45，P = 0.002）的多因素Cox回归模型中，这种效应仍具有统计学意义（HR 4.27，P = 0.009）。
高ABC反映疾病活动度更高及疾病增殖潜能更强。ABC和AMC独立预测生存期，因此似乎反映了不同的潜在病理生理过程。因此，两者都有可能改善当前的预后评分。
嗜碱性粒细胞是PMF恶性克隆的一部分，与不良疾病特征及预后不良相关，这独立于目前已确立的预后评分系统和单核细胞增多症。
