Functional platelet defects in children with severe chronic ITP as tested with 2 novel assays applicable for low platelet counts
| Autor: | Annemieke G. Laarhoven, Laila B. van der Heijden, Katja M. J. Heitink-Pollé, Mark Roest, C. Ellen van der Schoot, Gestur Vidarsson, Marrie C. A. Bruin, L. Porcelijn, Masja de Haas, Esther R. van Bladel, Philip G. de Groot |
|---|---|
| Přispěvatelé: | Landsteiner Laboratory, Clinical Haematology |
| Rok vydání: | 2014 |
| Předmět: |
Blood Platelets
Male Agonist Adolescent Platelet Aggregation Platelet Function Tests medicine.drug_class Immunology Biochemistry Pathogenesis Platelet degranulation medicine Humans Platelet Mean platelet volume Child Whole blood Autoimmune disease Purpura Thrombocytopenic Idiopathic Platelet Count business.industry Reproducibility of Results Convulxin Cell Biology Hematology Platelet Activation medicine.disease Child Preschool Tetradecanoylphorbol Acetate Female business |
| Zdroj: | Blood, 123(10), 1556-1563. American Society of Hematology |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood-2013-08-519686 |
| Popis: | Immune thrombocytopenia (ITP) is an autoimmune disease with a complex heterogeneous pathogenesis and a bleeding phenotype that is not necessarily correlated to platelet count. In this study, the platelet function was assessed in a well-defined cohort of 33 pediatric chronic ITP patients. Because regular platelet function test cannot be performed in patients with low platelet counts, 2 new assays were developed to determine platelet function: first, the microaggregation test, measuring in platelets isolated from 10 mL of whole blood the platelet potential to form microaggregates in response to an agonist; second, the platelet reactivity assay, measuring platelet reactivity to adenosine diphosphate (ADP), convulxin (CVX), and thrombin receptor activator peptide in only 150 mu L of unprocessed whole blood. Patients with a severe bleeding phenotype demonstrated a decreased aggregation potential upon phorbol myristate acetate stimulation, decreased platelet degranulation following ADP stimulation, and a higher concentration of ADP and CVX needed to activate the glycoprotein IIbIIIa complex compared with patients with a mild bleeding phenotype. In conclusion, here we have established 2 functional tests that allow for evaluation of platelet function in patients with extremely low platelet counts ( |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|