Szudy-Szczyrek Aneta, Mlak Radosław, Pigoń-Zając Dominika, Krupski Witold, Mazurek Marcin, Tomczak Aleksandra, Chromik Karolina, Górska Aleksandra, Koźlik Paweł, Juda Adrian, Kokoć Anna, Dubaj Maciej, Sacha Tomasz, Niedoszytko Marek, Helbig Grzegorz, Szczyrek Michał, Szumiło Justyna, Małecka-Massalska Teresa, Hus Marek
Department of Haematooncology and Bone Marrow Transplantation, Medical University of Lublin, Lublin, Staszica Street 11, 20-081, Poland.
Department of Laboratory Diagnostics, Medical University of Lublin, Lublin, Poland.
Sci Rep. 2025 Jan 2;15(1):161. doi: 10.1038/s41598-024-83851-0.
Mastocytosis is a heterogeneous group of disorders, characterized by accumulation of clonal mast cells which can infiltrate several organs, most often spine (70%). The pathogenesis of mastocytosis bone disease is poorly understood. The main aim of the study was to investigate whether neoplastic mast cells may be the source of sclerostin and whether there is an association between sclerostin and selected bone remodeling markers with mastocytosis related bone disease. We assessed sclerostin, bioactive sclerostin, and SOST gene expression in HMC-1.2 human mast cell culture supernatants and plasma of SM patients (n = 39). We showed that human mast cells can secrete sclerostin, and after their stimulation with IL-6, there is a significant increase in SOST gene expression. We observed significantly higher levels of sclerostin in patients diagnosed with more advanced disease. We observed a statistically significant correlation between concentations of sclerostin and its bioactive form and the concentration of alkaline phosphatase (ALP), and between sclerostin and interleukin-6 (IL-6). We observed that significantly higher sclerostin concentrations are present in patients with increased sclerosis of the spongy bone. Sclerostin may serve as a marker of more advanced disease and bone disease in mastocytosis. Further studies are justified to evaluate its role in mastocytosis.
肥大细胞增多症是一组异质性疾病，其特征为克隆性肥大细胞积聚，这些细胞可浸润多个器官，最常见于脊柱（70%）。肥大细胞增多症骨病的发病机制尚不清楚。本研究的主要目的是调查肿瘤性肥大细胞是否可能是硬化蛋白的来源，以及硬化蛋白与选定的骨重塑标志物之间是否与肥大细胞增多症相关骨病存在关联。我们评估了HMC-1.2人肥大细胞培养上清液和系统性肥大细胞增多症（SM）患者（n = 39）血浆中的硬化蛋白、生物活性硬化蛋白和SOST基因表达。我们发现人肥大细胞可分泌硬化蛋白，在用白细胞介素-6（IL-6）刺激后，SOST基因表达显著增加。我们观察到诊断为更晚期疾病的患者中硬化蛋白水平显著更高。我们观察到硬化蛋白及其生物活性形式的浓度与碱性磷酸酶（ALP）浓度之间以及硬化蛋白与白细胞介素-6（IL-6）之间存在统计学显著相关性。我们观察到松质骨硬化增加的患者中硬化蛋白浓度显著更高。硬化蛋白可能作为肥大细胞增多症中更晚期疾病和骨病的标志物。有必要进行进一步研究以评估其在肥大细胞增多症中的作用。
