Pérez-Cruz Fabiel Gerardo, Villa-Díaz Patricia, Pintado-Delgado María Consuelo, Fernández Rodríguez María Loreto, Blasco-Martínez Ana, Pérez-Fernández María
Fabiel Gerardo Pérez-Cruz, Internal Medicine Department, Hospital Universitario Príncipe de Asturias, Carretera Alcalá-Meco SN, 28805 Madrid, Spain.
World J Crit Care Med. 2017 May 4;6(2):135-139. doi: 10.5492/wjccm.v6.i2.135.
Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by platelet aggregation and mechanical damage to erythrocytes, clinically characterized by microangiopatic haemolytic anemia, thrombocytopenia and organ injury. We are reporting a case of a woman patient with severe hemolytic uremic syndrome associated to infectious diarrhoea caused by Shiga toxin-producing pathogen, who were admitted to our intensive care unit. The patient described developed as organ injury, neurological failure and acute renal failure, with need of haemodialysis technique. Due to the severity of the case and the delay in the results of the additional test that help us to the final diagnose, we treated her based on a syndromic approach of TMA with plasma exchange, with favourable clinical evolution with complete recovery of organ failures. We focus on the syndromic approach of these diseases, because thrombotic thrombocytopenic purpura, one of the disorders that are included in the syndromes of TMA, is considered a haematological urgency given their high mortality without treatment; and also review the TMA in adults: Their pathogenesis, management and outcomes.
血栓性微血管病(TMA)是一种微血管闭塞性疾病,其特征为血小板聚集和红细胞机械性损伤,临床特征为微血管性溶血性贫血、血小板减少和器官损伤。我们报告一例女性患者,患有与产志贺毒素病原体引起的感染性腹泻相关的严重溶血尿毒综合征,该患者入住了我们的重症监护病房。该患者出现了器官损伤、神经功能衰竭和急性肾衰竭,需要血液透析技术。由于病例的严重性以及有助于最终诊断的附加检查结果延迟,我们基于TMA的综合征方法对她进行了血浆置换治疗,临床进展良好,器官功能衰竭完全恢复。我们关注这些疾病的综合征方法,因为血栓性血小板减少性紫癜是TMA综合征所包含的疾病之一,由于其未经治疗死亡率高,被视为血液学急症;同时还回顾了成人TMA:其发病机制、管理和结局。