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John Goldman Fellowships for Future Science
Authors:Imane Agouti  Sylvie Cointe  Stéphane Robert  Coralie Judicone  Anderson Loundou  Fathi Driss  Alain Brisson  Dominique Steschenko  Christian Rose  Corinne Pondarré  Emmanuelle Bernit  Catherine Badens  Françoise Dignat‐George  Romaric Lacroix  Isabelle Thuret
Affiliation:1. Centre de Référence Maladies Rares Thalassémies, Marseille‐Lyon, Service d'Hémato‐Oncologie Pédiatrique, H?pital de la Timone, APHM, Marseille, France;2. VRCM, INSERM UMR‐S1076, UFR de Pharmacie, Université Aix Marseille, Marseille, France;3. Service d'Hématologie et de Biologie Vasculaire, H?pital de La Conception, AP‐HM, Marseille, France;4. RetT departement, Biocytex, Marseille, France;5. Unité d'Aide Méthodologique à la Recherche Clinique, AP‐HM, Marseille, France;6. Département de Biochimie, H?pital Bichat, AP‐HP, Paris, France;7. UMR‐CBMN, University of Bordeaux‐CNRS‐IPB, Pessac, France;8. Service d'Hémato‐Oncologie Pédiatrique, H?pital d'Enfants, CHU de Nancy, Nancy, France;9. Department d'Hématologie, H?pital Saint Vincent de Paul, Lille, France;10. Institut d'Hématologie et d'Oncologie Pédiatrique, Lyon, France;11. Service de Médecine Interne, H?pital de la conception, AP‐HM, Marseille, France
Abstract:The level of circulating platelet‐, erythrocyte‐, leucocyte‐ and endothelial‐derived microparticles detected by high‐sensitivity flow cytometry was investigated in 37 β‐thalassaemia major patients receiving a regular transfusion regimen. The phospholipid procoagulant potential of the circulating microparticles and the microparticle‐dependent tissue factor activity were evaluated. A high level of circulating erythrocyte‐ and platelet‐microparticles was found. In contrast, the number of endothelial microparticles was within the normal range. Platelet microparticles were significantly higher in splenectomized than in non‐splenectomized patients, independent of platelet count (< 0·001). Multivariate analysis indicated that phospholipid‐dependent procoagulant activity was influenced by both splenectomy (= 0·001) and platelet microparticle level (< 0·001). Erythrocyte microparticles were not related to splenectomy, appear to be devoid of proper procoagulant activity and no relationship between their production and haemolysis, dyserythropoiesis or oxidative stress markers could be established. Intra‐microparticle labelling with anti‐HbF antibodies showed that they originate only partially (median of 28%) from thalassaemic erythropoiesis. In conclusion, when βthalassaemia major patients are intensively transfused, the procoagulant activity associated with thalassaemic erythrocyte microparticles is probably diluted by transfusions. In contrast, platelet microparticles, being both more elevated and more procoagulant, especially after splenectomy, may contribute to the residual thrombotic risk reported in splenectomized multi‐transfused β‐thalassaemia major patients.
Keywords:β‐thalassaemia major  splenectomy  platelets‐derived microparticles  erythrocytes derived‐microparticles
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