Clinical Pediatrics, Hospital de Niños Dr. Ricardo Gutiérrez, City of Buenos Aires, Argentina.
Arch Argent Pediatr. 2023 Oct 1;121(5):e202202758. doi: 10.5546/aap.2022-02758.eng. Epub 2023 Feb 9.
Thrombotic thrombocytopenic purpura is a rare disease in pediatrics, but it has a high mortality if not managed in an adequate and timely manner. It is characterized by microangiopathic hemolytic anemia associated with neurological, cardiac, abdominal, and less frequently, renal signs and symptoms; it may be accompanied by fever. In children, diagnosis is based on clinical and laboratory findings. ADAMTS13 activity < 10% supports the diagnosis but does not confirm it and, given its severity, the result should not delay treatment initiation. Here we describe the case of a previously healthy 15-year-old female patient with neurological signs associated with hemolytic anemia and thrombocytopenia. During hospitalization, she was diagnosed with acquired thrombotic thrombocytopenic purpura.
血栓性血小板减少性紫癜在儿科中较为罕见，但如果不能及时、充分地治疗，其死亡率很高。其特征为微血管性溶血性贫血合并神经、心脏、腹部，以及较少见的肾脏症状和体征；可能伴有发热。在儿童中，诊断基于临床和实验室发现。ADAMTS13 活性＜10%支持诊断但不能确诊，且鉴于其严重程度，不应因等待结果而延迟治疗开始。在此我们描述了一例此前健康的 15 岁女性患者，她出现了神经症状，伴有溶血性贫血和血小板减少。住院期间，她被诊断为获得性血栓性血小板减少性紫癜。
