非典型溶血性尿毒综合征(HUS)在成人中极为罕见。HUS的特点是微血管病性溶血性贫血的标志性特征,血小板减少症,和急性肾损伤。非典型HUS(aHUS)是由不受控制的补体激活引起的。补体激活可由感染如肺炎链球菌或流感触发。怀孕,恶性肿瘤,细胞毒性药物,器官移植,或自身免疫性疾病。已发现基因突变和自身抗体在补体活性失调的发病机理中起关键作用。大多数由侵袭性肺炎链球菌感染引起的非典型HUS病例更常见于儿童。我们介绍了一例肺炎链球菌HUS(Sp-HUS),表现为多器官衰竭,弥散性血管内凝血(DIC),成人肢体缺血.该病例强调了在成人血栓性微血管病(TMA)的鉴别诊断中考虑肺炎链球菌HUS(Sp-HUS)的重要性。
Atypical hemolytic
uremic syndrome (HUS) is extremely rare in adults. HUS is characterized by hallmark features of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal injury. Atypical HUS (aHUS) is caused by uncontrolled complement activation. The complement activation can be triggered by infections such as Streptococcus pneumoniae or influenza, pregnancy, malignancy, cytotoxic drugs, organ transplants, or autoimmune diseases. Genetic mutations and autoantibodies have been found to play a crucial role in the pathogenesis of dysregulated complement activity. The majority of cases of atypical HUS due to invasive S. pneumoniae infection are more commonly seen in children. We present a
case of S. pneumoniae HUS (Sp-HUS) presenting with multiorgan failure, disseminated intravascular coagulation (DIC), and limb ischemia in an adult. This
case highlights the importance of considering S. pneumoniae HUS (Sp-HUS) in the differential diagnosis of thrombotic microangiopathies (TMA) in adults.