Šefer D, Bižić-Radulović S, Kraguljac-Kurtović N, Bogdanović A, Čokić V, Miljić P, Beleslin-Čokić B, Knežević V, Mitrović-Ajtić O, Leković D, Gotić M
Outpatient Clinic and Diagnostic Department, Clinic of Hematology, Clinical Center of Serbia, Belgrade, Serbia.
Medical Faculty University of Belgrade, Belgrade, Serbia.
Int J Lab Hematol. 2017 Feb;39(1):21-31. doi: 10.1111/ijlh.12564. Epub 2016 Dec 26.
Philadelphia-negative myeloproliferative neoplasms (Ph MPN) are characterized by overproduction of one or more blood cell lines.
We studied the proliferative characteristics of 91 patients with de novo Ph MPN. Colony-forming cells (CFC) and endogenous colonies (EC), from bone marrow (BM) and/or peripheral blood (PB), were analyzed by colony assay based on methylcellulose. The level of circulating CD34 cells was determined by flow cytometry.
The total number of PB CFC in primary myelofibrosis (PMF) was increased compared to the control sample (P < 0.01) and essential thrombocythemia (ET) (P < 0.05). The highest number of BM and PB EC was observed in polycythemia vera (PV) (P < 0.01). Increased levels of CD34 cells characterized early-prefibrotic (57%) and advanced-fibrotic PMF (90%) as compared to PV (34%) and ET (32%) (P < 0.01). In the whole Ph MPN group, the total number of PB CFC (P < 0.01), PB EC (P < 0.05), and CD34 cells (P < 0.01) correlated with the degree of BM fibrosis. Higher levels of circulating CD34 cells in PMF correlated with the total number of PB EC (P < 0.05) and degree of BM fibrosis (P < 0.01).
Exploration of the PB proliferative characteristics of Ph MPN on diagnosis may be helpful in revealing early-prefibrotic PMF. Monitoring the levels of circulating CD34 cells may provide a sensitive indicator of fibrotic evolution in PV and PMF.
费城染色体阴性骨髓增殖性肿瘤（Ph MPN）的特征是一种或多种血细胞系过度增殖。
我们研究了91例初发Ph MPN患者的增殖特性。通过基于甲基纤维素的集落分析法分析来自骨髓（BM）和/或外周血（PB）的集落形成细胞（CFC）和内源性集落（EC）。通过流式细胞术测定循环CD34细胞水平。
与对照样本（P < 0.01）和原发性血小板增多症（ET）（P < 0.05）相比，原发性骨髓纤维化（PMF）中PB CFC的总数增加。真性红细胞增多症（PV）中观察到BM和PB EC的数量最多（P < 0.01）。与PV（34%）和ET（32%）相比，早期纤维化前期（57%）和晚期纤维化PMF（90%）中CD34细胞水平升高（P < 0.01）。在整个Ph MPN组中，PB CFC总数（P < 0.01）、PB EC（P < 0.05）和CD34细胞（P < 0.01）与BM纤维化程度相关。PMF中循环CD34细胞水平较高与PB EC总数（P < 0.05）和BM纤维化程度（P < 0.01）相关。
在诊断时探索Ph MPN的PB增殖特性可能有助于揭示早期纤维化前期PMF。监测循环CD34细胞水平可能为PV和PMF的纤维化演变提供敏感指标。
