Abstract:
Secondary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition caused by the hyperactivation of macrophages and T-cells, triggered by infection, malignancy, or underlying rheumatological conditions. It rarely presents as a first manifestation of a rheumatological condition. Macrophage activation syndrome (MAS) is secondary HLH associated with underlying hematological conditions. Here, we present a case of a previously healthy 29-year-old female who was admitted with fever, rash, and pancytopenia, found to have HLH, and a workup revealed underlying systemic lupus erythematosus (SLE). She was successfully treated with dexamethasone, etoposide, and belimumab, with complete recovery of her symptoms. This case highlights the importance of a thorough evaluation of rheumatological conditions in all patients with HLH despite their previous medical history and the use of belimumab for SLE.
摘要:
继发性噬血细胞性淋巴组织细胞增生症(HLH)是由巨噬细胞和T细胞的过度活化引起的危及生命的高炎症状态,由感染引发,恶性肿瘤,或潜在的风湿病。它很少表现为风湿病的第一表现。巨噬细胞活化综合征(MAS)是与潜在血液学病症相关的继发性HLH。这里,我们介绍了一例以前健康的29岁女性,她因发烧入院,皮疹,和全血细胞减少症,发现有HLH,和检查显示潜在的系统性红斑狼疮(SLE)。她用地塞米松成功治疗,依托泊苷,还有belimumab,症状完全恢复.该病例强调了彻底评估所有HLH患者的风湿病的重要性,尽管他们以前的病史和使用贝利木单抗治疗SLE。