Rheumatology Department, Sheffield Children's Hospital, Sheffield, UK.
Paediatric and Adolescent Rheumatology, Sheffield Children's Hospital, Sheffield, UK.
Rheumatology (Oxford). 2019 Jan 1;58(1):5-17. doi: 10.1093/rheumatology/key006.
Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which if not promptly treated, can lead rapidly to critical illness and death. HLH is termed macrophage activation syndrome (MAS) when associated with rheumatic disease (where it is best characterized in systemic JIA) and secondary HLH (sHLH) when associated with other triggers including malignancy and infection. MAS/sHLH is rare and coupled with its mimicry of other conditions, is underrecognized. These inherent challenges can lead to diagnostic and management challenges in multiple medical specialties including haematology, infectious diseases, critical care and rheumatology. In this review we highlight the pathogenesis of MAS/sHLH including its underlying triggers, key clinical features and diagnostic challenges, prognostic factors and current treatments in adults.
噬血细胞性淋巴组织细胞增生症（HLH）是一种炎症过度活跃的综合征，如果不及时治疗，可能会迅速导致重病和死亡。当与风湿性疾病相关时，HLH 被称为巨噬细胞活化综合征（MAS）（在全身性幼年特发性关节炎中表现最为明显），当与其他触发因素相关时，如恶性肿瘤和感染，则被称为继发性 HLH（sHLH）。MAS/sHLH 较为罕见，并且由于其与其他疾病的相似性，往往被漏诊。这些内在的挑战可能会导致血液科、传染病科、重症监护科和风湿科等多个医学专业在诊断和治疗方面面临挑战。在这篇综述中，我们重点介绍了 MAS/sHLH 的发病机制，包括其潜在的触发因素、关键临床特征和诊断挑战、预后因素以及成人目前的治疗方法。
