Effects of eltrombopag on platelet count and platelet activation in Wiskott-Aldrich syndrome/X-linked thrombocytopenia

Author:

Gerrits Anja J.1,Leven Emily A.2,Frelinger Andrew L.1,Brigstocke Sophie L.2,Berny-Lang Michelle A.1,Mitchell W. Beau23,Revel-Vilk Shoshana4,Tamary Hannah5,Carmichael Sabrina L.1,Barnard Marc R.1,Michelson Alan D.1,Bussel James B.2

Affiliation:

1. Center for Platelet Research Studies, Division of Hematology/Oncology, Boston Children’s Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA;

2. Division of Pediatric Hematology/Oncology, New York Presbyterian Hospital/Weill Cornell Medical College, New York, NY;

3. Platelet Biology Laboratory, New York Blood Center, New York, NY;

4. Department of Pediatric Hematology/Oncology, Hadassah Hebrew University Hospital, Jerusalem, Israel; and

5. Pediatric Hematology Oncology, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel

Abstract

Key Points Platelet function in WAS/XLT, measured by agonist-induced surface-activated GPIIb-IIIa and P-selectin, is proportional to platelet size. Eltrombopag increased platelet counts, but did not improve platelet activation, in most WAS/XLT patients.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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