Glanzmann's thrombasthenia associated with gastrointestinal angiodysplasias successfully treated with bevacizumab.

IF 1.2 4区 医学 Q4 HEMATOLOGY
Blood Coagulation & Fibrinolysis Pub Date : 2023-12-01 Epub Date: 2023-10-20 DOI:10.1097/MBC.0000000000001249
Agustina Saladino, María L Gonzalez, Fernando A Chuliber, Marcelo M Serra
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引用次数: 0

Abstract

Glanzmann's Thrombasthenia (GT) is a rare hemorrhagic condition caused by a platelet surface receptor disorder of the glycoprotein (GP) IIb/IIIa. Symptoms of GT are various forms of hemorrhages, such as purpura, epistaxis and menorrhagia. Gastrointestinal bleeding (GIB) is a rare expression of the condition and may occur due to traumas in the GI tract or as a consequence of gastrointestinal angiodysplasia (GIADs). In this case report, we present a middle-aged woman with recurrent GIB consequent to GIADs with persistent melena and iron deficiency anemia. After several unsuccessful therapeutic interventions, the patient was studied by the hereditary hemorrhagic telangiectasia's (HHT - Osler-Weber-Rendu disease) unit, where she received bevacizumab, showing a complete improvement in symptoms as well as a reduction in her GIADs. This case shows that bevacizumab could be a possible line of treatment for patients with coagulation disorders with GIADs.

贝伐单抗成功治疗与胃肠道血管发育不良相关的Glanzmann血栓衰弱。
Glanzmann氏血栓衰弱(GT)是一种罕见的出血性疾病,由糖蛋白(GP)IIb/IIIa的血小板表面受体紊乱引起。GT的症状是各种形式的出血,如紫癜、鼻出血和月经过多。胃肠道出血(GIB)是这种情况的一种罕见表现,可能是由于胃肠道创伤或胃肠道血管发育不良(GIAD)引起的。在本病例报告中,我们报告了一名中年妇女,她因患有持续性黑便和缺铁性贫血的GIAD而复发性GIB。在几次治疗干预失败后,患者接受了遗传性出血性毛细血管扩张症(HHT-Osler-Weber-Rendu病)单位的研究,在那里她接受了贝伐单抗治疗,症状完全改善,GIAD减少。该病例表明,贝伐单抗可能是治疗GIAD凝血障碍患者的一种可能途径。
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来源期刊
CiteScore
1.90
自引率
0.00%
发文量
111
审稿时长
4-8 weeks
期刊介绍: Blood Coagulation & Fibrinolysis is an international fully refereed journal that features review and original research articles on all clinical, laboratory and experimental aspects of haemostasis and thrombosis. The journal is devoted to publishing significant developments worldwide in the field of blood coagulation, fibrinolysis, thrombosis, platelets and the kininogen-kinin system, as well as dealing with those aspects of blood rheology relevant to haemostasis and the effects of drugs on haemostatic components
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