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[Platelet function after allogeneic bone marrow transplantation in Wiskott-Aldrich syndrome]
M Megido1, J M Cesar, M S Maldonado
1Servicio de Hematología, Hospital Ramón y Cajal, Madrid.
Purpose:
To assess the platelet characteristics and functionalism in the Wiskott-Aldrich syndrome (WAS) after allogeneic BMT using cyclophosphamide and busulphan for conditioning.
Material And Methods:
Two WAS patients underwent allogeneic BMT. Platelet aggregation was studied prior to and after BMT, along with the intraplatelet amount of ADP and ATP.
Results:
Platelet count, size and aggregation wholly recovered after BMT. The post-transplant content of platelet nucleotides was normal.
Conclusions:
Platelet function can be totally restored with cyclophosphamide/busulphan conditioned BMT in WAS. Platelet defects in this disease are due to defective thrombopoiesis.