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沙利度胺治疗 Glanzmann 血小板无力症严重复发性肠血管发育不良出血的长期疗效。

Long-term treatment with thalidomide for severe recurrent hemorrhage from intestinal angiodysplasia in Glanzmann Thrombasthenia.

机构信息

Department of Medicine, Section of Internal and Cardiovascular Medicine, University of Perugia, Perugia, Italy.

Division of Internal Medicine and Division of Immunology and Transfusion, Mazzoni Hospital, Ascoli Piceno, Italy.

出版信息

Platelets. 2021 Feb 17;32(2):288-291. doi: 10.1080/09537104.2020.1745169. Epub 2020 Mar 21.

DOI:10.1080/09537104.2020.1745169
PMID:32200672
Abstract

Gastrointestinal angiodysplasia (GIA) is the most common cause of occult gastrointestinal bleeding (GIB) requiring often hospitalization and transfusions, especially in patients with hemorrhagic disorders. Thalidomide, impairing neo-angiogenesis, has been successfully used in the management of bleeding in patients with GIA and in particular in patients with inherited bleeding disorders. Only one case of short-term treatment with thalidomide in a patient with Glanzmann thrombasthenia (GT) and recurrent GIB due to GIA has been reported so far.We report the case of a woman with GT developing high frequency recurrent GIB due to GIA requiring repeated blood and platelet transfusions, who was treated with thalidomide obtaining a striking and stable reduction of GIB and of the requirement of platelet and blood transfusions for over 5 years. Moreover, we raise the suspicion that the association between GT and GIA may not be fortuitous.

摘要

胃肠道血管发育不良(GIA)是最常见的需要经常住院和输血的隐匿性胃肠道出血(GIB)的原因,尤其是在有出血性疾病的患者中。抑制新血管生成的沙利度胺已成功用于治疗 GIA 患者的出血,特别是用于治疗遗传性出血性疾病患者的出血。迄今为止,仅报道了一例因 GIA 导致 Glanzmann 血小板无力症(GT)和复发性 GIB 的患者短期沙利度胺治疗的病例。我们报告了一例 GT 患者因 GIA 导致高频复发性 GIB 而需要反复输血和血小板输注,该患者接受沙利度胺治疗后,GIB 和血小板输注的需求显著且稳定减少,超过 5 年。此外,我们怀疑 GT 和 GIA 之间的关联可能并非偶然。

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