title: Secondary macrophage activation syndrome due to autoimmune, hematologic, infectious and oncologic diseases. Thirteen case series and review of the literature.
abstract: 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.
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España. Electronic address: tonoeguesdubuc@hotmail.com.
authors.forename: César
authors.initials: C
authors.lastname: Egües Dubuc
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors.forename: Vicente
authors.initials: V
authors.lastname: Aldasoro Cáceres
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors.forename: Miren
authors.initials: M
authors.lastname: Uriarte Ecenarro
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors.forename: Nerea
authors.initials: N
authors.lastname: Errazquin Aguirre
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors.forename: Iñaki
authors.initials: I
authors.lastname: Hernando Rubio
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors.forename: Carlos Francisco
authors.initials: CF
authors.lastname: Meneses Villalba
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors.forename: Esther
authors.initials: E
authors.lastname: Uriarte Itzazelaia
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors.forename: Jorge J
authors.initials: JJ
authors.lastname: Cancio Fanlo
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors.forename: Olga
authors.initials: O
authors.lastname: Maiz Alonso
authors.affiliation: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors.forename: Joaquin M
authors.initials: JM
authors.lastname: Belzunegui Otano
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000150:complications* / Q000175:diagnosis; D018450:Disease Progression; D005260:Female; D006402:Hematologic Diseases / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007239:Infections / Q000150:complications* / Q000175:diagnosis; D055501:Macrophage Activation Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000150:complications* / Q000175:diagnosis; D011379:Prognosis
keywords: Células hemofagocíticas; Hemofagocitosis; Hemophagocytic cells; Hemophagocytic syndrome; Hemophagocytosis; Linfohistiocitocis hemofagocítica; Linfohistiocitocis hemophagocytic; Macrophage activation syndrome; Síndrome de activación macrofágica; Síndrome hemofagocítico