Hematology Division, Osmangazi University Medical School, Meşelik Campus, Eskişehir, Turkey.
Blood Coagul Fibrinolysis. 2021 Jul 1;32(5):349-351. doi: 10.1097/MBC.0000000000001026.
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.
血管性血友病是一种遗传性疾病，由于血小板功能障碍导致出血发作。血小板输注是中重度出血的标准治疗方法。重组因子 VIIa（rFVIIa）在出血发作和侵入性操作中成功应用。在此，我们介绍一名血管性血友病患者，其出血发作只能用 rFVIIa 控制。患者为 28 岁男性，经常出血发作，对局部止血剂和氨甲环酸无反应，并对血小板输注发生过敏反应。我们开始每周给患者使用低剂量（20μg/kg）rFVIIa。从那时起，患者没有自发性出血。这是首例常规低剂量 rFVIIa 预防血管性血友病患者出血的病例报告。
