Ahmad Anam, Atluri Rama, Robbins Katherine J
Internal Medicine, St. Luke's Hospital, Missouri, USA.
Rheumatology, St. Louis University, St. Louis, USA.
Cureus. 2024 Jul 15;16(7):e64596. doi: 10.7759/cureus.64596. eCollection 2024 Jul.
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.
继发性噬血细胞性淋巴组织细胞增生症（HLH）是一种由巨噬细胞和T细胞过度激活引起的危及生命的高炎症状态，由感染、恶性肿瘤或潜在的风湿性疾病引发。它很少作为风湿性疾病的首发表现。巨噬细胞活化综合征（MAS）是与潜在血液系统疾病相关的继发性HLH。在此，我们报告一例既往健康的29岁女性病例，该患者因发热、皮疹和全血细胞减少入院，诊断为HLH，进一步检查发现潜在的系统性红斑狼疮（SLE）。她接受地塞米松、依托泊苷和贝利尤单抗治疗后成功康复，症状完全缓解。该病例强调了对所有HLH患者进行全面风湿性疾病评估的重要性，无论其既往病史如何，以及贝利尤单抗在SLE治疗中的应用。
