Krecak Ivan, Gveric-Krecak Velka, Roncevic Pavle, Basic-Kinda Sandra, Gulin Josipa, Lapic Ivana, Fumic Ksenija, Ilic Ivana, Horvat Ivana, Zadro Renata, Holik Hrvoje, Coha Bozena, Peran Nena, Aurer Igor, Durakovic Nadira
a Department of Internal Medicine , General Hospital of Sibenik-Knin County , Sibenik , Croatia.
b Division of Hematology, Department of Internal Medicine , University Hospital Center Zagreb , Zagreb , Croatia.
Hematology. 2018 Dec;23(10):793-802. doi: 10.1080/10245332.2018.1498157. Epub 2018 Jul 11.
Serum chitotriosidase activity (CHIT1) is a biomarker of macrophage activation with an important role in inflammation-induced tissue remodeling and fibrosis. Macrophages have been described to play a crucial role in regulating pathological erythropoiesis in polycythemia vera (PV). The aim of this study was to evaluate CHIT1 in patients diagnosed with Philadelphia-negative myeloproliferative neoplasms (MPNs).
Using fluorometric assay, we measured CHIT1 in 28 PV, 27 essential thrombocythemia (ET), 17 primary myelofibrosis (PMF), 19 patients with secondary myelofibrosis and in 25 healthy controls.
CHIT1 was significantly higher in PV (p < .001) and post-PV myelofibrosis (MF) transformation (post-PV MF) (p = .020), but not in ET (p = .080), post-ET MF transformation (p = .086), and PMF patients (p = .287), when compared to healthy controls. CHIT1 in PV was positively correlated with hemoglobin (p = .026), hematocrit (p = .012), absolute basophil count (p = .030) and the presence of reticulin fibrosis in the bone marrow (p = .023).
A positive correlation between CHIT1 and these distinct laboratory PV features might imply macrophages closely related to clonal erythropoiesis as cells of CHIT1 origin. In addition, a positive association between CHIT1 and reticulin fibrosis might indicate its potential role in PV progression.
CHIT1 might be considered as a circulating biomarker in PV. Additional studies are needed to clarify the role of CHIT1 in promoting disease progression and bone marrow fibrosis in PV.
血清壳三糖苷酶活性(CHIT1)是巨噬细胞活化的生物标志物,在炎症诱导的组织重塑和纤维化中起重要作用。巨噬细胞在真性红细胞增多症(PV)中调节病理性红细胞生成方面发挥关键作用。本研究旨在评估诊断为费城染色体阴性骨髓增殖性肿瘤(MPN)患者的CHIT1水平。
采用荧光测定法,我们检测了28例PV患者、27例原发性血小板增多症(ET)患者、17例原发性骨髓纤维化(PMF)患者、19例继发性骨髓纤维化患者以及25名健康对照者的CHIT1水平。
与健康对照相比,PV患者(p < 0.001)和PV后骨髓纤维化(MF)转化患者(p = 0.020)的CHIT1水平显著升高,但ET患者(p = 0.080)、ET后MF转化患者(p = 0.086)和PMF患者(p = 0.287)的CHIT1水平未升高。PV患者的CHIT1与血红蛋白(p = 0.026)、血细胞比容(p = 0.012)、绝对嗜碱性粒细胞计数(p = 0.030)以及骨髓中网硬蛋白纤维化的存在(p = 0.023)呈正相关。
CHIT1与这些不同的PV实验室特征之间的正相关可能意味着巨噬细胞与克隆性红细胞生成密切相关,是CHIT1的来源细胞。此外,CHIT1与网硬蛋白纤维化之间的正相关可能表明其在PV进展中的潜在作用。
CHIT1可能被视为PV的循环生物标志物。需要进一步研究以阐明CHIT1在促进PV疾病进展和骨髓纤维化中的作用。