Track topics on Twitter Track topics that are important to you
Inherited platelet disorders are rare bleeding syndromes due to either platelet function abnormalities or thrombocytopenia which may be associated with functional defects. The haemorrhagic symptoms observed in these patients are mostly muco-cutaneous and of highly variable severity. Although 30 to 50% of the platelet disorders are still of unknown origin, the precise diagnosis of these pathologies by specialized laboratories together with haemorrhagic scores enables an assessment of the risk of bleeding in each patient. Depending on the diagnostic elements collected, an appropriate medical procedure can be proposed for each situation: scheduled or emergency surgical interventions and pregnancy follow-up. The pathologies most at risk correspond to Glanzmann's thrombasthenia, Bernard-Soulier syndrome, severe thrombocytopenia (<40,000 platelets/μL) and signalling protein abnormalities affecting the activation of GPIIb-IIIa, a membrane glycoprotein essential for platelet aggregation. For these particular patients, in whom the risk of bleeding can be increased by a factor of 40, management protocols during surgical procedures are generally based on the use of conventional platelet concentrates, for both prophylaxis and the control of active bleeding. The perinatal period in women with platelet disorders and their new-born also require special attention. Indeed, beyond unpredictable delivery haemorrhages, bleeding requiring a blood transfusion is observed after delivery in more than 50% of women with Glanzmann's thrombastenia or Bernard-Soulier syndrome.
This article was published in the following journal.
Name: Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine
Platelets are a critical component for effecting hemostasis and wound healing. Disorders affecting any platelet pathway mediating adhesion, activation, aggregation and procoagulant surface exposure ca...
The laboratory diagnostics of (inherited) platelet function disorders mainly comprises aggregation and secretion assays, which may be suitable for diagnosing some specific severe platelet function dis...
Treatment decisions for patients with immune thrombocytopenia (ITP) are difficult because patients with similarly low platelet counts differ in their bleeding tendency. We recently reported that pl...
The prevalence of bleeding disorders, notably von Willebrand disease (vWD), among adult women with objectively documented menorrhagia is consistently reported to be 10% to 20% and is even higher in ad...
The number of genes involved in the identification of macrothrombocytopenia (MTP) is growing but the clinical consequences for the affected patients are not well determined. Here, we report the manage...
It seems reasonable to assume that patients who present significant bleeding symptoms may have different quality of platelets than those without bleeding. This question was addressed in a ...
Blood contains red blood cells, white blood cells, and platelets, as well as a fluid portion termed plasma. We primarily study blood platelets, but sometimes we also analyze the blood of p...
The primary objective of this study is to compare the three study arms of lower, medium, and higher dose platelet therapy with respect to the percentage of patients experiencing at least o...
Prospective cohort study on adult cardiac surgery patients. Platelet function evaluated before and after cardiopulmonary bypass. Bleeding and transfusion recorded. Primary endpoint is find...
Acute upper gastrointestinal bleeding (AUGIB) is a common medical emergency. In an ageing population, antiplatelet drugs are increasingly being prescribed for treatment and prophylaxis aga...
A rare, inherited platelet disorder characterized by a selective deficiency in the number and contents of platelet alpha-granules. It is associated with THROMBOCYTOPENIA, enlarged platelets, and prolonged bleeding time.
Group of hemorrhagic disorders in which the VON WILLEBRAND FACTOR is either quantitatively or qualitatively abnormal. They are usually inherited as an autosomal dominant trait though rare kindreds are autosomal recessive. Symptoms vary depending on severity and disease type but may include prolonged bleeding time, deficiency of factor VIII, and impaired platelet adhesion.
A congenital bleeding disorder with prolonged bleeding time, absence of aggregation of platelets in response to most agents, especially ADP, and impaired or absent clot retraction. Platelet membranes are deficient in or have a defect in the glycoprotein IIb-IIIa complex (PLATELET GLYCOPROTEIN GPIIB-IIIA COMPLEX).
The process of minimizing risk to an organization by developing systems to identify and analyze potential hazards to prevent accidents, injuries, and other adverse occurrences, and by attempting to handle events and incidents which do occur in such a manner that their effect and cost are minimized. Effective risk management has its greatest benefits in application to insurance in order to avert or minimize financial liability. (From Slee & Slee: Health care terms, 2d ed)
Disorders caused by abnormalities in platelet count or function.
Obstetrics and gynaecology
Fertility Menopause Obstetrics & Gynaecology Osteoporosis Women's Health Obstetrics and gynaecology comprises the care of the pregnant woman, her unborn child and the management of diseases specific to women. Most consultant...