Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.clml.2013.10.004 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Catheter Intervention in a Patient with Intracranial Aneurysms and Glanzmann Thrombasthenia Caused by a Novel Homozygous Likely Pathogenic Variant in the Gene.
Diseases
June 2024
Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, Medical Center-University of Freiburg, 79106 Freiburg, Germany.
Glanzmann Thrombasthenia (GT) is an inherited platelet disorder caused by defects in platelet integrin αβ (GPIIb/IIIa), which is a platelet receptor essential for the binding of fibrinogen. This can lead to severe bleeding, especially after trauma or perioperatively, and to microcytic anemia because of chronic blood loss. We report on a 40-year-old female patient with extensive bleeding complications and platelet antibody formation who presented in Homburg and Freiburg for extensive platelet function analyses and molecular genetic analyses.
View Article and Find Full Text PDFPatient with a history of Glanzmann thrombasthenia presented with chronic subdural hematoma: a case report study.
Oxf Med Case Reports
February 2024
Independent researcher, Shiraz, Iran.
Glanzmann thrombasthenia (GT) is a rare platelet disorder characterized by qualitative/quantitative deficiencies of the platelets' fibrinogen receptor, glycoprotein (GP) IIb/IIIa complex, resulting in impaired platelet aggregation and increased bleeding time. Most cases are hereditary with an autosomal recessive pattern of inheritance, but acquired GT also occurs. We report the surgical management of symptomatic chronic subdural hematoma (CSDH), a rare condition in young individuals, in a 37-year-old man who had GT and a history of mild head trauma approximately one month before admission.
View Article and Find Full Text PDFAcquired Glanzmann's thrombasthenia with IgG and IgA against activated α β.
Br J Haematol
September 2023
Department of Haematology, University College London Hospitals NHS Foundation Trust, London, UK.
Current status and future prospects of activated recombinant coagulation factor VIIa, NovoSeven®, in the treatment of haemophilia and rare bleeding disorders.
Ann Hematol
August 2024
Thrombosis and Hemostasis Research Center, Nara Medical University, 840 Shijo‑cho, Kashihara City, Nara, 634-8522, Japan.
Article Synopsis
- rFVIIa is a human recombinant activated coagulation factor VII used for over two decades to treat and prevent bleeding in various bleeding disorders, including congenital and acquired hemophilia, congenital factor VII deficiency, and Glanzmann thrombasthenia.
- Its dosage, administration, and indications vary between the US, Europe, and Japan based on patient needs and regulatory practices.
- Despite the emergence of new therapies like emicizumab for hemophilia A, rFVIIa remains crucial for managing bleeding episodes, especially in surgical situations and when other treatments fail.
Introduction Glanzmann's thrombasthenia (GT) is an autosomal recessive disorder of platelets caused by a deficiency in the glycoprotein IIb-IIIa. Bleeding from the skin, mucous membranes, and ecchymosis are symptoms manifested starting in early childhood. There may also be major bleeding conditions as a result of surgical procedures or trauma.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!