Normalized levels of red blood cells expressing phosphatidylserine, their microparticles, and activated platelets in young patients with β-thalassemia following bone marrow transplantation

Ann Hematol. 2017 Oct;96(10):1741-1747. doi: 10.1007/s00277-017-3070-2. Epub 2017 Jul 26.

Abstract

Bone marrow transplantation (BMT) serves as the only curative treatment for patients with β-thalassemia major; however, hemostatic changes have been observed in these BMT patients. Aggregability of thalassemic red blood cells (RBCs) and increased red blood cell-derived microparticles (RMPs) expressing phosphatidylserine (PS) are thought to participate in thromboembolic events by initially triggering platelet activation. To our knowledge, there has been no report providing quantitation of these circulating PS-expressing RBCs and RMPs in young β-thalassemia patients after BMT. Whole blood from each subject was fluorescently labeled to detect RBC markers (CD235a) and annexin-V together with the known number TruCount™ beads. PS-expressing RBCs, RMPs, and activated platelets were identified by flow cytometry. In our randomized study, we found the decreased levels of three aforementioned factors compared to levels in patients receiving regular blood transfusion (RT). This study showed that BMT in β-thalassemia patients decreases the levels of circulating PS-expressing RBCs, their MPs, and procoagulant platelets when compared to patients who received RT. Normalized levels of these coagulation markers may provide the supportive evidence of the effectiveness of BMT for curing thalassemia.

Keywords: Activated platelets; Beta-thalassemia; Bone marrow transplantation; Microparticles; Phosphatidylserine-expressing red blood cells.

Publication types

  • Clinical Trial

MeSH terms

  • Adolescent
  • Allografts
  • Annexin A5 / blood
  • Blood Platelets / metabolism*
  • Bone Marrow Transplantation*
  • Cell-Derived Microparticles / metabolism*
  • Child
  • Erythrocytes / metabolism*
  • Female
  • Humans
  • Male
  • Phosphatidylserines / blood*
  • Platelet Activation*
  • beta-Thalassemia* / blood
  • beta-Thalassemia* / therapy

Substances

  • Annexin A5
  • Phosphatidylserines