Platelets compensate for poor thrombin generation in type 3 von Willebrand disease

Szántó, Tímea; Nummi, Vuokko; Jouppila, Annukka; Brinkman, Herm Jan M.; Lassila, Riitta

doi:10.1080/09537104.2019.1581922

Cited by 13 publications

(18 citation statements)

References 33 publications

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“…The significant role of platelets in coagulation was shown when measuring thrombin generation (TG) in plasma of VWD3 patients (absent VWF in plasma, platelet and endothelial cells) [175]. Although TG was markedly impaired in the plasma of VWD3 patients, it was close to normal in the presence of platelets [176]. The importance of platelets in coagulation is also clear in congenital FV deficiency (Owren parahemophilia), a rare bleeding disorder (the incidence is 1:1.000.000) inherited in an autosomal recessive trait [177].…”

Section: Bleeding and The Interplay Between Platelets And Coagulationmentioning

confidence: 99%

Interplay between platelets and coagulation

et al. 2021

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Elsevier has created a COVID-19 resource centre with free information in English and Mandarin on the novel coronavirus COVID-19. The COVID-19 resource centre is hosted on Elsevier Connect, the company's public news and information website. Elsevier hereby grants permission to make all its COVID-19-related research that is available on the COVID-19 resource centre-including this research content-immediately available in PubMed Central and other publicly funded repositories, such as the WHO COVID database with rights for unrestricted research re-use and analyses in any form or by any means with acknowledgement of the original source. These permissions are granted for free by Elsevier for as long as the COVID-19 resource centre remains active.

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Section: Bleeding and The Interplay Between Platelets And Coagulationmentioning

confidence: 99%

Interplay between platelets and coagulation

et al. 2021

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“…overall comparable ETP and 13% decreased peak height in PRP-TG compared with healthy controls (84). The reason behind the discrepancy between ETP and peak height of PRP-TG in VWD patients is still unclear but Szanto et al (84) attribute the normal ETP in type 3 VWD to the compensatory role played by hyperresponsive platelets.…”

Section: Accepted Manuscriptmentioning

confidence: 99%

Added Value of Blood Cells in Thrombin Generation Testing

et al. 2021

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The capacity of blood to form thrombin is a critical determinant of coagulability. Plasma thrombin generation (TG), a test that probes the capacity of plasma to form thrombin, has improved our knowledge of the coagulation system and shows promising utility in coagulation management. Although plasma TG gives comprehensive insights in the function of pro- and anticoagulation drivers, it does not measure the role of blood cells in TG. In this literature review, we discuss currently available continuous TG tests that can reflect the involvement of blood cells in coagulation, in particular the fluorogenic assays that allow continuous measurement in platelet rich plasma and whole blood. We also provide an overview about the influence of blood cells on blood coagulation, with emphasis on the direct influence of blood cells on TG. Platelets accelerate the initiation and velocity of thrombin generation by phosphatidylserine exposure, granule content release and surface receptor interaction with coagulation proteins. Erythrocytes are also major providers of phosphatidylserine and erythrocyte membranes trigger contact activation. Furthermore, leukocytes and cancer cells may be important players in cell-mediated coagulation, because under certain conditions, they express tissue factor, release procoagulant components and can induce platelet activation. We argue that testing TG in the presence of blood cells may be useful to distinguish blood cells-related coagulation disorders. However, it should also be noted that these blood cells-dependent TG assays are not clinically validated. Further standardization and validation studies are needed to explore their clinical usefulness.

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“…Several case series have shown that platelet transfusions can improve haemostasis and may stop bleeding episodes in VWD patients (Fressinaud et al , ; Castillo et al , ). Recent in vitro studies indicated that platelets restore thrombin generation in VWD type 3 (Szanto et al , ). No clinical data are available yet to indicate when platelet transfusions should be initiated in severe VWD patients with intractable bleeding.…”

Section: Supportive Treatment Of Severe Vwdmentioning

confidence: 99%

How I manage severe von Willebrand disease

Leebeek¹,

Atiq²

2019

Br J Haematol

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Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Most patients with mild and moderate VWD can be treated effectively with desmopressin. The management of severe VWD patients, mostly affected by type 2 and type 3 disease, can be challenging. In this article we review the current diagnosis and treatment of severe VWD patients. We will also discuss the management of severe VWD patients in specific situations, such as pregnancy, delivery, patients developing alloantibodies against von Willebrand factor and VWD patients with recurrent gastrointestinal bleeding. Moreover, we review emerging treatments that may be applied in future management of patients with severe VWD.

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Platelets compensate for poor thrombin generation in type 3 von Willebrand disease

Cited by 13 publications

References 33 publications

Interplay between platelets and coagulation

Interplay between platelets and coagulation

Added Value of Blood Cells in Thrombin Generation Testing

How I manage severe von Willebrand disease

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