Advertisement

Topics

Inherited platelet disorders : Management of the bleeding risk.

08:00 EDT 1st August 2018 | BioPortfolio

Summary of "Inherited platelet disorders : Management of the bleeding risk."

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.

Affiliation

Journal Details

This article was published in the following journal.

Name: Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine
ISSN: 1953-8022
Pages:

Links

DeepDyve research library

PubMed Articles [25664 Associated PubMed Articles listed on BioPortfolio]

Iron deficiency and anemia in patients with inherited bleeding disorders.

Patients with inherited bleeding disorders are predisposed to acute and chronic blood loss, which places them at high risk of iron deficiency anemia (IDA). The clinical effects of iron deficiency (ID)...

Inherited platelet functional disorders: General principles and practical aspects of management.

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...

Management of pregnancy for a patient with the new syndromic macrothrombocytopenia, DIAPH1-related disease.

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...

Spinal Epidural Bleed in Hemophilia: A Rare Site of Bleeding With Complete Resolution With Nonsurgical Management.

Hemophilia A is an inherited bleeding disorder, seen in 1 in 10,000 live births. It is characterized by an increased risk of bleeding, especially involving the musculoskeletal system. Central nervous ...

Anesthetic management without perioperative platelet transfusion for cervical laminectomy and laminoplasty in a case of May-Hegglin anomaly.

May-Hegglin anomaly (MHA) is an inherited autosomal dominant disorder characterized by giant platelets and inclusion bodies in granulocytes, and thrombocytopenia. There is no consensus on the perioper...

Clinical Trials [12149 Associated Clinical Trials listed on BioPortfolio]

Platelet Glycoproteins in Inherited Thrombocytopathy: Association With Aggregation Studies and Bleeding Severity

Disorders of platelet function are characterized by variable mucocutaneous bleeding manifestations and excessive hemorrhage following surgical procedures or trauma. Generally, most patient...

The Association Between Platelet Reactivity and Bleeding Risk in Adult ITP

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 ...

Perioperative Eltrombopag in Patients With Inherited Thrombocytopenia

The objective of the study is to estimate the response to eltrombopag based on platelet count increase above a safety level of 80 G/L and lack of requirement for pre-, per- and post-operat...

The Genetics and Functional Basis of Inherited Platelet, White Blood Cell, Red Blood Cell, and Blood Clotting Disorders.

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...

Optimal Platelet Dose Strategy for Management of Thrombocytopenia

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...

Medical and Biotech [MESH] Definitions

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.

Advertisement
Quick Search
Advertisement
Advertisement

 


DeepDyve research library

Relevant Topics

Surgical treatments
Surgery is a technology consisting of a physical intervention on tissues. All forms of surgery are considered invasive procedures; so-called "noninvasive surgery" usually refers to an excision that does not penetrate the structure being exci...

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...


Searches Linking to this Article