Erythrocytes from patients with myeloproliferative neoplasms and splanchnic venous thrombosis show greater expression of Lu/BCAM

被引:5
|
作者
Novitzky-Basso, I. [1 ,2 ]
Spring, F. [3 ]
Anstee, D. [3 ]
Tripathi, D. [4 ,5 ]
Chen, F. [2 ,5 ,6 ]
机构
[1] Queen Elizabeth Univ Hosp, Scottish Blood & Marrow Transplant Unit, Glasgow, Lanark, Scotland
[2] Queen Elizabeth Hosp Birmingham NHS Trust, Ctr Clin Haematol, Birmingham, W Midlands, England
[3] NHS Blood & Transplant, Bristol, Avon, England
[4] Queen Elizabeth Hosp Birmingham NHS Trust, Liver Unit, Birmingham, W Midlands, England
[5] Univ Birmingham, Ctr Rare Dis, Birmingham, W Midlands, England
[6] NHS Blood & Transplant, Birmingham, W Midlands, England
关键词
Budd-Chiari syndrome; JAK-2 Protein Tyrosine Kinase; Lutheran blood group system; myeloproliferative disorders; thrombosis; BLOOD-GROUP GLYCOPROTEIN; POLYCYTHEMIA-VERA; ESSENTIAL THROMBOCYTHEMIA; VEIN-THROMBOSIS; GROUP ANTIGENS; CELL ADHESION; MUTATION; JAK2; ERYTHROPOIESIS; MANAGEMENT;
D O I
10.1111/ijlh.12838
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
IntroductionLutheran/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. MethodsWe 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. ResultsLu 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. ConclusionsPatients 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.
引用
收藏
页码:473 / 477
页数:5
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