title: Platelet function tests, independent of platelet count, are associated with bleeding severity in ITP.
abstract: Immune thrombocytopenia (ITP) patients with similarly low platelet counts differ in their tendency to bleed. To determine if differences in platelet function in ITP patients account for this variation in bleeding tendency, we conducted a single-center, cross-sectional study of pediatric patients with ITP. Bleeding severity (assessed by standardized bleeding score) and platelet function (assessed by whole blood flow cytometry) with and without agonist stimulation was evaluated in 57 ITP patients (median age, 9.9 years). After adjustment for platelet count, higher levels of thrombin receptor activating peptide (TRAP)-stimulated percent P-selectin- and activated glycoprotein (GP)IIb-IIIa-positive platelets were significantly associated with a lower bleeding score, whereas higher levels of immature platelet fraction (IPF), TRAP-stimulated platelet surface CD42b, unstimulated platelet surface P-selectin, and platelet forward light scatter (FSC) were associated with a higher bleeding score. Thus, platelet function tests related to platelet age (IPF, FSC) and activation through the protease activated receptor 1 (PAR1) thrombin receptor (TRAP-stimulated P-selectin, activated GPIIb-IIIa, and CD42b), independent of platelet count, are associated with concurrent bleeding severity in ITP. These tests may be useful markers of future bleeding risk in ITP.
authors.affiliation: Center for Platelet Research Studies, and Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
authors.forename: Andrew L
authors.initials: AL
authors.lastname: Frelinger
authors.affiliation: Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
authors.forename: Rachael F
authors.initials: RF
authors.lastname: Grace
authors.affiliation: Center for Platelet Research Studies, and Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
authors.forename: Anja J
authors.initials: AJ
authors.lastname: Gerrits
authors.affiliation: Center for Platelet Research Studies, and Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
authors.forename: Michelle A
authors.initials: MA
authors.lastname: Berny-Lang
authors.affiliation: Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
authors.forename: Travis
authors.initials: T
authors.lastname: Brown
authors.affiliation: Center for Platelet Research Studies, and Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
authors.forename: Sabrina L
authors.initials: SL
authors.lastname: Carmichael
authors.affiliation: Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
authors.forename: Ellis J
authors.initials: EJ
authors.lastname: Neufeld
authors.affiliation: Center for Platelet Research Studies, and Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
authors.forename: Alan D
authors.initials: AD
authors.lastname: Michelson
mesh_terms: D000293:Adolescent; D001792:Blood Platelets / Q000473:pathology / Q000502:physiology; D002454:Cell Differentiation; D055252:Cell-Derived Microparticles / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D005434:Flow Cytometry; D006470:Hemorrhage / Q000097:blood* / Q000209:etiology*; D006801:Humans; D008027:Light; D008297:Male; D063847:Mean Platelet Volume; D019007:P-Selectin / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D010976:Platelet Count; D010979:Platelet Function Tests; D019039:Platelet Glycoprotein GPIIb-IIIa Complex / Q000378:metabolism; D019038:Platelet Glycoprotein GPIb-IX Complex / Q000378:metabolism; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood* / Q000150:complications*; D044463:Receptor, PAR-1 / Q000097:blood; D012542:Scattering, Radiation
chemical_list: D019007:P-Selectin; D010446:Peptide Fragments; D019039:Platelet Glycoprotein GPIIb-IIIa Complex; D019038:Platelet Glycoprotein GPIb-IX Complex; D044463:Receptor, PAR-1; C086309:thrombin receptor peptide SFLLRNP