血栓性血小板减少性紫癜(TTP)是一种罕见的微血管病性疾病,有时与系统性红斑狼疮(SLE)有关。然而,这种可能的因果关系尚未得到完全证实。两种疾病的诊断区分首先是困难的,因为它们具有可能重叠的相似特征。我们介绍了一例32岁女性,自12岁起就患有癫痫发作。病人因头痛的临床表现被送往急诊室,发烧,皮肤和粘膜苍白,困惑的状态,感觉异常,和四肢短暂性痉挛.实验室结果显示Coombs直接阴性溶血性贫血,严重的血小板减少症,乳酸脱氢酶显著升高,并在外围膜中存在分裂细胞++。此外,阳性抗核抗体和阳性抗天然DNA,滴度分别为1/320和1/160,被发现了。尿液分析显示血肌酐正常。由于医院资源有限,未评估ADAMTS13。然而,基于临床,血液学,和生化发现,我们得出的结论是,这是一例与SLE相关的TTP,并表明采用血浆置换和甲基强的松龙脉冲治疗,获得令人满意的反应(生物标志物水平的正常化,健康状况)在第二次血浆置换后。SLE和TTP的诊断通常很难实现;然而,临床表现和实验室检查的充分相关性,在部分治疗干预的帮助下,可能导致良好的临床反应。
Thrombotic thrombocytopenic purpura (TTP) is an uncommon microangiopathic disease and sometimes is associated with systemic lupus erythematous (SLE). However, this probable causal relationship has not been completely proven. The diagnostic differentiation of both diseases is difficult in the first instance because they share similar characteristics that may overlap. We present a
case of a 32-year-old woman with antecedents of epileptic seizures since she was 12 years old. The patient was admitted to the emergency room with a clinical picture of headaches, fever, paleness in the skin and mucosa, confused state, paresthesia, and transient spasticity of the extremities. The laboratory results revealed direct Coombs negative hemolytic anemia, severe thrombocytopenia, significant elevation of lactate dehydrogenase, and presence of schistocytes ++ in the peripheral film. In addition, positive antinuclear antibodies and positive anti-native DNA in titers of 1/320 and 1/160, respectively, were found. Urinalysis showed that serum creatinine was in normal range. Because of limited hospital resources, ADAMTS13 was not evaluated. However, based on clinical, hematological, and biochemical findings, we concluded that it was a
case of TTP associated with SLE and indicated treatment with plasmapheresis and methylprednisolone pulses, obtaining a satisfactory response (normalization of biomarker levels, health condition) after the second session of plasmapheresis. Diagnosis of both SLE and TTP is often difficult to achieve; however, adequate correlation of clinical manifestations and laboratory tests, along with the help of partial therapeutic interventions, may lead to good clinical response.