Scottish Blood and Marrow Transplant Unit, Queen Elizabeth University Hospital, Glasgow, UK.
NHS Blood and Transplant, Filton, Bristol, UK.
Int J Lab Hematol. 2018 Aug;40(4):473-477. doi: 10.1111/ijlh.12838. Epub 2018 May 13.
Lutheran/BCAM protein (Lu) on the surface of erythrocytes is key for their adhesion to the endothelium, and erythrocytes from individuals with JAK2V617F-mutated myeloproliferative neoplasms (MPN) have increased endothelial adhesion. Splanchnic vein thrombosis (SVT) is a devastating thrombotic complication of MPN, and frequently, the only diagnostic feature is the JAK2V617F mutation. We sought to examine whether erythrocytes from patients with JAK2V617F mutated SVT (MPN-SVT) exhibited increased Lu expression, thereby supporting a mechanistic contribution to the development of thrombosis.
We report the validation of a novel flow cytometry assay for Lu expression on erythrocytes. We examined the expression of Lu on erythrocytes from a cohort of MPN patients with and without SVT, and healthy controls. Samples were obtained from 20 normal individuals, 22 with MPN (both JAK2V617F-mutated and wild-type) and 8 with JAK2V617F-mutated MPN-SVT.
Lu expression by erythrocytes from patients with MPN and MPN-SVT is significantly increased compared to erythrocytes from healthy individuals (P < .05), but there was no significant difference between patients with MPN-SVT and MPN.
Patients with MPN have increased expression of the red cell Lu/BCAM adhesion molecule. Further work is required to determine the role of the increased Lu/BCAM adhesion to the endothelium in the development of thrombosis in MPN of all genotypes.
红细胞表面的吕克/BCAM 蛋白(Lu)是其与内皮细胞黏附的关键,而 JAK2V617F 突变骨髓增殖性肿瘤(MPN)患者的红细胞具有增强的内皮黏附性。脾静脉血栓形成(SVT)是 MPN 的一种破坏性血栓并发症,通常唯一的诊断特征是 JAK2V617F 突变。我们试图研究 JAK2V617F 突变 SVT(MPN-SVT)患者的红细胞是否表现出 Lu 表达增加,从而支持其在血栓形成发展中的机制贡献。
我们报告了一种用于红细胞上 Lu 表达的新型流式细胞术检测的验证。我们检查了来自 MPN 患者伴或不伴 SVT 和健康对照者的红细胞上 Lu 的表达。从 20 名正常个体、22 名 MPN(JAK2V617F 突变和野生型)和 8 名 JAK2V617F 突变 MPN-SVT 患者中获得样本。
与健康个体相比,MPN 和 MPN-SVT 患者的红细胞上 Lu 的表达显著增加(P <.05),但 MPN-SVT 患者与 MPN 患者之间无显著差异。
MPN 患者的红细胞 Lu/BCAM 黏附分子表达增加。需要进一步研究以确定所有基因型 MPN 中增加的 Lu/BCAM 与内皮黏附在血栓形成发展中的作用。