Pubfacts - Scientific Publication Data
  • Categories
  • |
  • Journals
  • |
  • Authors
  • Login
  • Categories
  • Journals

Search Our Scientific Publications & Authors

Publications
  • Publications
  • Authors
find publications by category +
Translate page:

Functional platelet defects in children with severe chronic ITP as tested with 2 novel assays applicable for low platelet counts.

Authors:
Esther R van Bladel Annemieke G Laarhoven Laila B van der Heijden Katja M Heitink-Pollé Leendert Porcelijn C Ellen van der Schoot Masja de Haas Mark Roest Gestur Vidarsson Philip G de Groot Marrie C A Bruin

Blood 2014 Mar 2;123(10):1556-63. Epub 2014 Jan 2.

Department of Clinical Chemistry and Hematology, University Medical Center Utrecht, Utrecht, The Netherlands;

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 μL 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 (<10(9)). These tests show that platelet function is related to bleeding phenotype in chronic ITP.

Download full-text PDF

Source
http://dx.doi.org/10.1182/blood-2013-08-519686DOI Listing
March 2014

Publication Analysis

Top Keywords

platelet function
16
platelet
12
platelet counts
12
bleeding phenotype
12
low platelet
12
platelet reactivity
8
chronic itp
8
patients
5
diphosphate adp
4
adenosine diphosphate
4
convulxin cvx
4
adp convulxin
4
thrombin receptor
4
unprocessed blood
4
blood patients
4
patients severe
4
μl unprocessed
4
150 μl
4
receptor activator
4
activator peptide
4

Similar Publications

The Influence of Lipid Excipients on Platelet Function and the Pharmacodynamic Effects of Aspirin.

Authors:
David J Schneider Heidi S Taatjes-Sommer Jayne Prats Efthymios N Deliargyris

J Cardiovasc Pharmacol 2021 Feb 19. Epub 2021 Feb 19.

Departments of Medicine, Cardiovascular Research Institute, The University of Vermont, Burlington, Vermont.

Abstract: The combination of pharmaceutical lipid excipients with aspirin in a novel liquid oral formulation (Vazalore) limits gastrointestinal toxicity of aspirin. This study was performed to determine whether the lipid excipients influence the pharmacodynamic effects of aspirin and determine whether the excipients directly affect platelet function. The pharmacodynamic effects of aspirin were assessed over a range of concentrations designed to exert limited to maximal inhibition of cyclooxygenase-1 (COX1) necessary for thromboxane A2 production. Read More

View Article and Full-Text PDF
February 2021
Similar Publications

A homozygous loss-of-function mutation in GP1BB causing variable clinical phenotypes in a family with Bernard-Soulier syndrome.

Authors:
Nouf Al-Numair Khushnooda Ramzan Laila Alquait Meshal Alshehri Faiqa Imtiaz Tarek Owaidah

Blood Coagul Fibrinolysis 2021 Mar 1. Epub 2021 Mar 1.

Centre for Genomic Medicine, King Faisal Specialist Hospital and Research Centre College of Medicine, Alfaisal University Department of Pathology and Laboratory Medicine, Centre of Excellence Thrombosis and Homeostasis, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

Bernard-Soulier syndrome is a rare autosomal recessive bleeding disorder and has a low incidence. Bernard-Soulier syndrome is caused by the deficiency of glycoprotein GPIb-V-IX complex, a receptor for von Willebrand factor and is characterized by thrombocytopenia, giant platelets and bleeding tendency. We are reporting three members of a same family with variable phenotypic clinical presentation. Read More

View Article and Full-Text PDF
March 2021
Similar Publications

HLA-haploidentical TCRαβ+/CD19+-depleted stem cell transplantation in children and young adults with Fanconi anemia.

Authors:
Luisa Strocchio Daria Pagliara Mattia Algeri Giuseppina Li Pira Francesca Rossi Valentina Bertaina Giovanna Leone Rita Maria Pinto Marco Andreani Emanuele Agolini Katia Girardi Stefania Gaspari Lavinia Grapulin Francesca Del Bufalo Antonio Novelli Pietro Merli Franco Locatelli

Blood Adv 2021 Mar;5(5):1333-1339

Department of Pediatric Hemato-Oncology and Cell and Gene Therapy, Scientific Institute for Research and Healthcare (IRCCS) Bambino Gesù Children's Hospital, Rome, Italy.

We report on the outcome of 24 patients with Fanconi anemia (FA) lacking an HLA matched related or unrelated donor, given an HLA-haploidentical T-cell receptor αβ (TCRαβ+) and CD19+ cell-depleted hematopoietic stem cell transplantation (HSCT) in the context of a prospective, single-center phase 2 trial. Sustained primary engraftment was achieved in 22 (91.6%) of 24 patients, with median time to neutrophil recovery of 12 days (range, 9-15 days) and platelet recovery of 10 days (range, 7-14 days). Read More

View Article and Full-Text PDF
March 2021
Similar Publications

Straight Channel Microfluidic Chips for the Study of Platelet Adhesion under Flow.

Authors:
Alexander Dupuy Lining Arnold Ju Freda H Passam

Bio Protoc 2019 Mar 20;9(6):e3195. Epub 2019 Mar 20.

Heart Research Institute, Newtown, NSW 2042, Australia.

Microfluidic devices have become an integral method of cardiovascular research as they enable the study of shear force in biological processes, such as platelet function and thrombus formation. Furthermore, microfluidic chips offer the benefits of testing of platelet adhesion using small amounts of blood or purified platelets. Microfluidic chips comprise flow channels of varying dimensions and geometries which are connected to a syringe pump. Read More

View Article and Full-Text PDF
March 2019
Similar Publications

Bilateral serous retinal detachment: an unusual complication of HELLP syndrome.

Authors:
Obaid Imtiyazul Haque Abdul Waris Syed Asghar Rizvi

BMJ Case Rep 2021 Mar 2;14(3). Epub 2021 Mar 2.

Institute of Ophthalmology, Jawaharlal Nehru Medical College & Hospital, Aligarh, Uttar Pradesh, India.

A 23-year-old primigravida presented to the emergency department with reports of headache. On examination, her blood pressure was found to be 190/140 mm Hg. Her laboratory results were notable for proteinuria, deranged liver function and low platelets. Read More

View Article and Full-Text PDF
March 2021
Similar Publications
© 2021 PubFacts.
  • About PubFacts
  • Privacy Policy
  • Sitemap