Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin
Flávio Sztajnbok, Adriana Rodrigues Fonseca, Leonardo Rodrigues Campos, Kátia Lino, Marta Cristine Félix Rodrigues, Rodrigo M. Silva, Rozana Gasparello de Almeida, Sandro Félix Perazzio, Margarida de Fátima Fernandes Carvalho
Abstract
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.