Sattler Laurent, Wimmer Jordan, Herb Agathe, Gerout Anne-Cécile, Feugeas Olivier, Desprez Dominique
Laboratoire d'Hématologie, Unité Hémostase, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
Service de pharmacie, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
Res Pract Thromb Haemost. 2023 May 20;7(4):100183. doi: 10.1016/j.rpth.2023.100183. eCollection 2023 May.
Glanzmann thrombasthenia (GT) is a rare congenital platelet function disorder associated with a severe bleeding diathesis. Thrombotic manifestations remain a rare condition. We report here the first case of recurrent venous thromboembolism (VTE) successfully treated with apixaban in a patient with GT. Our patient's morbid obesity was an additional challenge.
The Key Clinical Question was to determine if direct oral anticoagulants are suitable for patients with both obesity and GT.
In our patient, the first episode of VTE occurred after the use of a low dose of activated recombinant factor VII for a minor procedure, whereas the second was unprovoked. Administration of rivaroxaban very quickly led to the appearance of bleeding symptoms and subsequently led to poor compliance and extension of deep vein thrombosis. The patient was switched to apixaban, with very good efficacy and safety over the cumulative 18 months of use.
The last updated guidelines now recommend the use of rivaroxaban and apixaban for management of VTE in patients with obesity. Regarding patients with GT, there is still insufficient data on the use of direct oral anticoagulants. Management of thrombotic manifestations in these patients remains a rare and complex condition and could be improved by the creation of a specific international registry.
Glanzmann血小板无力症(GT)是一种罕见的先天性血小板功能障碍,伴有严重的出血素质。血栓形成表现仍然罕见。我们在此报告首例复发性静脉血栓栓塞(VTE)患者,该患者患有GT,使用阿哌沙班成功治疗。我们的患者病态肥胖是另一个挑战。
关键临床问题是确定直接口服抗凝剂是否适用于肥胖且患有GT的患者。
在我们的患者中,首次VTE发作发生在使用低剂量活化重组因子VII进行小手术后,而第二次发作是自发的。使用利伐沙班很快导致出血症状出现,随后导致依从性差和深静脉血栓形成扩展。患者改用阿哌沙班,在累计使用的18个月中疗效和安全性非常好。
最新指南现在推荐使用利伐沙班和阿哌沙班治疗肥胖患者的VTE。对于患有GT的患者,关于直接口服抗凝剂的使用仍缺乏足够的数据。这些患者血栓形成表现的管理仍然罕见且复杂,创建一个特定的国际登记处可能会改善这种情况。