Weekly low-dose recombinant factor VIIa prophylaxis in Glanzmann thrombasthenia

Autor: Hava Üsküdar Teke, Neslihan Andic, Tuba Bulduk, Eren Gündüz, Nur Oğuz
Rok vydání: 2021
Předmět:
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