title: Intravenous immunoglobulin treatment for macrophage activation syndrome complicating chronic granulomatous disease.
abstract: OBJECTIVE
  Chronic granulomatous disease is a rare phagocyte disorder characterized by an increased susceptibility to infections and inflammatory complications. We describe two patients with chronic granulomatous disease (CGD) complicated by macrophage activation syndrome (MAS) (secondary hemophagocytic lymphohistiocytosis) treated with intravenous immunoglobulin (IVIG).
  
  
  METHODS
  A report of two cases of CGD complicated by MAS who were successfully treated with IVIG was made, and a comparison was made with ten other cases reported in the literature.
  
  
  RESULTS
  MAS is a severe potentially fatal complication of CGD. Most cases are associated with Burkholderia cepacia and leishmaniasis infection. The treatment of these patients varies between centers, and one example is the use of the HLH-2004 protocol. IVIG could be an effective first line option for this complication in CGD patients.
  
  
  CONCLUSIONS
  The exaggerated inflammatory response characteristic of CGD patients could play a role in the development of this complication. IVIG appears to be a safe and effective first line treatment in these patients.
authors: Clinical Immunology Department, Instituto Nacional de Pediatría, Insurgentes sur 3700-C, Insurgentes Cuicuilco Coyoacan CP, Mexico City, Mexico.
authors: Aristóteles
authors: A
authors: Álvarez-Cardona
authors: Ana Luisa
authors: AL
authors: Rodríguez-Lozano
authors: Lizbeth
authors: L
authors: Blancas-Galicia
authors: Francisco Eduardo
authors: FE
authors: Rivas-Larrauri
authors: Marco A
authors: MA
authors: Yamazaki-Nakashimada
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000235:genetics; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007223:Infant; D055501:Macrophage Activation Syndrome / Q000150:complications* / Q000628:therapy*; D008297:Male; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous