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Congenital Platelet Dysfunction and von Willebrand Disease
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
The underlying problem in Bernard-Soulier disease is a quantitative or qualitative abnormality in the platelet GP Ib/IX/V receptor complex for vWF. A single mutation in any of the individual glycoprotein chains comprising the receptor can result in a failure of the receptor to insert in the platelet membrane. Specifically, the Bernard-Soulier phenotype may result from either a homozygous or a doubly heterozygous mutation in GP Ib alpha, GP Ib beta, GP IX, or GP V. Additionally, a mutation in the leucine-rich region of GP Ib alpha expressed in an autosomal dominant fashion may result in a Bernard-Soulier phenotype (14, 15). Preparation of platelet-rich plasma may be unusually difficult in Bernard-Soulier disease, since the platelets in this disorder are characteristically of increased size and tend to be spun down with the leukocytes. Whole blood flow cytometric analysis may accordingly be of particular utility in the diagnosis of Bernard-Soulier disease (9).
Haemostasis: Normal Physiology, Disorders of Haemostasis and Thrombosis
Published in John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie, Basic Sciences Endocrine Surgery Rhinology, 2018
Elizabeth Jones, Russell David Keenan
This is the platelets sticking to exposed collagen underlying the damaged endothelium. There are two main membrane bound sticky molecular complexes that have this function: Glycoprotein IIb/IIIa complex, which sticks to collagen directly. If this glycoprotein is missing it causes a severe bleeding disorder called Glanzmann’s thrombasthenia.Glycoprotein Ib/V/IX complex, which sticks to collagen indirectly via von Willebrand factor. If this glycoprotein is missing it causes a severe bleeding disorder called Bernard Soulier disease.
Effects of bisphenol A and S on blood coagulation: in vivo, in vitro and in silico approaches in toxicodynamic
Published in Toxicology Mechanisms and Methods, 2021
Artur Paes Chagas, Beatriz Pereira Peixoto, Bianca Barros da Costa, Thamyris Almeida Moreira, Leonardo Paes Cinelli, Leandro Louback da Silva, Leandro Miranda-Alves, Clemilson Berto-Junior
As a very first step, treatment of zebrafish animals with increasing concentrations of both molecules and well-established bleeding inducers, as heparin and ASA. Recording bleeding time displayed an augment in time required to cease bleeding in 200 nM of BPA, when compared to control animals, with no effect in 4 and 40 nM. Considering BPS, none of the studied concentrations could modulate in vivo bleeding time. It is noticeable that bleeding time for control animals in our experiments showed similar values to literature (around 40 s) (Lang et al. 2010). Even though very simple, bleeding time record in zebrafish models is very reliable, since they possess great similarity with mammal’s homeostasis systems, and may be very useful to new drugs screen (Lang et al. 2010). Besides, the assay is widely used to evaluate platelet function in clinical practice, helping to diagnoses thrombocytopenia, von Willebrand disease, disseminated intravascular coagulation, Glanzmann’s thrombasthenia and Bernard-Soulier disease (Russeau and Manna 2018).