Flavio Sztajnbok, Adriana Rodrigues Fonseca, Leonardo Rodrigues Campos, Kátia Lino, Marta Cristine Félix Rodrigues, Rodrigo Moulin Silva, Rozana Gasparello de Almeida, Sandro Félix Perazzio, Margarida de Fátima Fernandes Carvalho
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引用次数: 0
摘要
嗜血细胞淋巴组织细胞增生症(HLH)是一种罕见的遗传性高炎症综合征,早年即会发病。巨噬细胞活化综合征(MAS)通常是指与自身免疫相关的继发性HLH,但继发性HLH也有其他病因,如感染和恶性肿瘤。本文回顾了 HLH 和 MAS 的概念、流行病学、临床和实验室特征、诊断、鉴别诊断、预后和治疗。我们还回顾了 MAS 在影响儿童的最常见自身免疫性疾病中的存在。这两种疾病都很严重,需要及时诊断和治疗,以避免发病和死亡。
Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin
Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.
期刊介绍:
Formerly named Revista Brasileira de Reumatologia, the journal is celebrating its 60th year of publication.
Advances in Rheumatology is an international, open access journal publishing pre-clinical, translational and clinical studies on all aspects of paediatric and adult rheumatic diseases, including degenerative, inflammatory and autoimmune conditions. The journal is the official publication of the Brazilian Society of Rheumatology and welcomes original research (including systematic reviews and meta-analyses), literature reviews, guidelines and letters arising from published material.