Intravenous immunoglobulin treatment for macrophage activation syndrome complicating chronic granulomatous disease.
J Clin Immunol
; 32(2): 207-11, 2012 Apr.
Article
em En
| MEDLINE
| ID: mdl-22119934
ABSTRACT
OBJECTIVES:
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Imunoglobulinas Intravenosas
/
Síndrome de Ativação Macrofágica
/
Doença Granulomatosa Crônica
Tipo de estudo:
Guideline
Limite:
Adolescent
/
Child
/
Child, preschool
/
Humans
/
Infant
/
Male
Idioma:
En
Ano de publicação:
2012
Tipo de documento:
Article