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. Hemophagocitc 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.
Keywords: Células hemofagocíticas; Hemofagocitosis; Hemophagocytic cells; Hemophagocytic syndrome; Hemophagocytosis; Linfohistiocitocis hemofagocítica; Linfohistiocitocis hemophagocytic; Macrophage activation syndrome; Síndrome de activación macrofágica; Síndrome hemofagocítico.
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