Caeiro Alves Filipa, Aguiar Rute, Pessegueiro Pedro, Pires Carlos
Department of Nephrology, Hospital do Espirito Santo de Évora EPE, Évora, Portugal.
BMJ Case Rep. 2018 Mar 17;2018:bcr-2017-222582. doi: 10.1136/bcr-2017-222582.
Thrombotic microangiopathy (TMA) comprises a process of sequential endothelial damage, microvascular thrombosis, consumptive thrombocytopenia and microangiopathic haemolytic anaemia that can affect several organs, including the kidney. A 36-year-old woman was presented with a petechial rash 3 weeks after an upper respiratory tract infection. Laboratory results showed normocytic normochromic anaemia, thrombocytopenia and evidence of TMA with decreased haptoglobin, elevated serum lactate dehydrogenase and a peripheral blood smear with numerous schistocytes. Treatment included daily plasmapheresis and prednisolone, with favourable clinical evolution. Antibodies anti-ADAMTS13 were positive, establishing the diagnosis of acquired thrombotic thrombocytopenic purpura. There was also serological evidence of a recent infection by , and therefore the preceding respiratory tract infection by this agent was the most likely trigger for the disease. Due to the high mortality rate and poor outcomes, the prompt diagnostic and treatment are crucial in this rare disease. The identification of triggers related to this pathology can allow new therapeutic targets or preventive strategies.
血栓性微血管病(TMA)是一个连续的内皮损伤、微血管血栓形成、消耗性血小板减少和微血管病性溶血性贫血的过程,可累及包括肾脏在内的多个器官。一名36岁女性在上呼吸道感染3周后出现瘀点皮疹。实验室检查结果显示正细胞正色素性贫血、血小板减少,且有TMA证据,表现为触珠蛋白降低、血清乳酸脱氢酶升高以及外周血涂片可见大量裂体细胞。治疗包括每日进行血浆置换和使用泼尼松龙,临床病情呈良好进展。抗ADAMTS13抗体阳性,确诊为获得性血栓性血小板减少性紫癜。也有血清学证据表明近期感染了[病原体名称未给出],因此该病原体先前引起的呼吸道感染最有可能是该疾病的触发因素。由于该疾病死亡率高且预后不佳,及时诊断和治疗在这种罕见病中至关重要。识别与该病理相关的触发因素可为新的治疗靶点或预防策略提供依据。