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Increased endothelial activation in α-thalassemia disease

  • Pankamol Sirivadhanakul
  • Ampaiwan Chuansumrit
  • Duantida Songdej
  • Praguywan Kadegasem
  • Pakawan Wongwerawattanakoon
  • Nongnuch SirachainanEmail author
Original Article
  • 26 Downloads

Abstract

One complication of thalassemia is thromboembolism (TE), which is caused by an abnormal red blood cell surface, as well as endothelial and platelet activation. These findings are commonly observed in severe β-thalassemia. However, limited information on α-thalassemia exists. This study enrolled subjects with deletional and non-deletional α-thalassemia and normal controls (NC). Plasma and serum of subjects were tested for endothelial activation markers including thrombomodulin (TM), vascular cell adhesion molecule-1 (VCAM-1), and von Willebrand factor antigen as well as platelet activation markers including thromboxane B2 and platelet factor 4. A total of 179 subjects were enrolled: 29 in the deletional group (mean age 13.3 ± 4.4 years), 31 in the non-deletional group (mean age 12.9 ± 4.8 years), and 119 in the NC group (mean age 13.6 ± 3.0 years). Twenty nine percent of subjects in the non-deletional group received regular red blood cell transfusion and iron chelator administration. Serum ferritin level was higher in the non-deletional group than that in the deletional group. Multivariate analysis demonstrated that VCAM-1 and TM levels were increased significantly in α-thalassemia compared with NC group (816.8 ± 131.0 vs 593.9 ± 49.0 ng/ml, and 4.9 ± 0.7 vs 4.0 ± 0.4 ng/ml, P < 0.001 respectively). VCAM-1 and TM levels in the non-deletional group were significantly higher than that in the deletional group. The present study demonstrated endothelial activation in children with α-thalassemia disease, especially those in the non-deletional group, which might be one risk factor for TE in α-thalassemia disease.

Keywords

α-Thalassemia Children Endothelial activation Thromboembolism 

Abbreviations

BMI

Body mass index

β-TG

β-Thromboglobulin

CBC

Complete blood count

CECs

Circulating endothelial cells

CS

Hb constant spring

DNA

Deoxyribonucleic acid

ELAM

E-selectin

F1.2

Prothrombin fragment 1 + 2

Hb

Hemoglobin

HPLC

High-performance liquid chromatography

ICAM-1

Intercellular adhesion molecule-1

MCH

Mean corpuscular hemoglobin

MCV

Mean corpuscular volume

NC

Normal controls

NTDT

Non-transfusion dependent thalassemia

PF4

Platelet factor 4

PS

Hb Paksé

RBC

Red blood cell

RDW

Red cell distribution width

TAT

Thrombin-antithrombin complex

TBX2

Thromboxane B2

TDT

Transfusion-dependent thalassemia

TE

Thromboembolism

TM

Thrombomodulin

VCAM-1

Vascular cell adhesion molecule-1

VEGF

Vascular endothelial growth factor

VWF:Ag

Von Willebrand factor antigen

Notes

Acknowledgements

NC designed the study and reviewed the manuscript. PS performed the study and wrote the manuscript. AC, DS, and PW involved in the care of patients. PK performed the laboratory.

Funding information

This work was supported by a Grant from the Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. We thank Edanz Group (www.edanzediting.com/ac) for editing a draft of this manuscript.

Compliance with ethical standards

Ethical approval for the study protocol was obtained from the Ethical Clearance Committee on Human Rights Related to Research to Research Involving Human Subjects, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Thailand (ID 12-59-09).

Conflict of interest

The authors declare that they have no conflicts of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Pediatrics, Faculty of Medicine Ramathibodi HospitalMahidol UniversityBangkokThailand
  2. 2.Department of Nursing, Faculty of Medicine Ramathibodi HospitalMahidol UniversityBangkokThailand

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