Secondary macrophage activation syndrome due to autoimmune, hematologic, infectious and oncologic diseases. Thirteen case series and review of the literature

Objective Describe the demographic characteristics and disorders of patients with diagnosis of macrophage activation syndrome (MAS) in the December 2008–January 2014 period. Methods Medical records were reviewed from diagnosis of MAS and after discharge until January 2014. Patients were divided into 4 groups according to the primary disease: autoimmune (AI), hemato-oncologic (HO), infectious (Inf) and oncologic (Onc). The variables were analyzed among the 4 groups and between AI and HO. Results Thirteen patients [7 men, with a median of 54 years (32–63)] were studied. The etiologies were: 5 AI, 5 HO, 2 Inf and 1 Onc disease. Hemophagocytic cells were found in the ascitic fluid of one patient. A patient with MAS secondary to IgG4-related disease was found. Conclusions Mortality, prognosis and disease progression may be influenced by the delay in diagnosis, treatment initiation and etiology of MAS. HO ill patients had a worse prognosis.