Weekly low-dose recombinant factor VIIa prophylaxis in Glanzmann thrombasthenia
Autor: | Hava Üsküdar Teke, Neslihan Andic, Tuba Bulduk, Eren Gündüz, Nur Oğuz |
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Rok vydání: | 2021 |
Předmět: |
Adult
Male congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Hemorrhage Factor VIIa Gastroenterology hemic and lymphatic diseases Internal medicine medicine Humans Hemostatic Agent Hematology biology Dose-Response Relationship Drug business.industry Standard treatment Low dose General Medicine Recombinant Proteins Platelet transfusion Recombinant factor VIIa Glanzmann thrombasthenia biology.protein business Tranexamic acid medicine.drug Thrombasthenia |
Zdroj: | Blood coagulationfibrinolysis : an international journal in haemostasis and thrombosis. 32(5) |
ISSN: | 1473-5733 |
Popis: | Glanzmann thrombasthenia is an inherited disease causing bleeding episodes due to platelet dysfunction. The standard treatment for moderate-severe bleeding is platelet transfusion. Recombinant factor VIIa (rFVIIa) is successfully used in bleeding episodes and invasive procedures. Here, we present a patient with Glanzmann thrombasthenia, whose bleeding episodes could only be controlled by rFVIIa. The patient is a 28 years old male, who has had frequent bleeding episodes unresponsive to local hemostatic agents and tranexamic acid and had an anaphylactoid reaction to platelet transfusion. We started the patient on a low-dose (20 μg/kg) rFVIIa once a week. The patient has no spontaneous bleeding since then. This is the first case report of a Glanzmann thrombasthenia patient on routine prophylaxis with low-dose rFVIIa. |
Databáze: | OpenAIRE |
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