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Vol. 11. Issue 3.
Pages 139-143 (May - June 2015)
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Vol. 11. Issue 3.
Pages 139-143 (May - June 2015)
Brief report
DOI: 10.1016/j.reumae.2014.06.006
Secondary Macrophage Activation Syndrome Due to Autoimmune, Hematologic, Infectious and Oncologic Diseases. Thirteen Case Series and Review of the Literature
Síndrome de activación macrofágica secundario a enfermedades autoinmunes, hematológicas, infecciosas y oncológicas. Serie de 13 casos clínicos y una revisión bibliográfica
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César Egües Dubuc
Corresponding author
tonoeguesdubuc@hotmail.com

Corresponding author.
, Vicente Aldasoro Cáceres, Miren Uriarte Ecenarro, Nerea Errazquin Aguirre, Iñaki Hernando Rubio, Carlos Francisco Meneses Villalba, Esther Uriarte Itzazelaia, Jorge J. Cancio Fanlo, Olga Maiz Alonso, Joaquin M. Belzunegui Otano
Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, Spain
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Tables (4)
Table 1. Demographic Characteristics, Etiologies, Triggers, Clinical Course, Treatment and Mortality of Patients With Secondary Macrophage Activation Syndrome.
Table 2. Descriptive Analysis of the Variables of Patients With Macrophage Activation Syndrome Secondary to Autoimmune Diseases.
Table 3. Descriptive Analysis of the Variables of Patients With Macrophage Activation Syndrome Secondary to Hemato-oncological Diseases.
Table 4. Descriptive Analysis of the Variables of Patients With Macrophage Activation Syndrome Secondary to Infectious and Oncological Diseases.
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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. 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.

Keywords:
Linfohistiocitocis hemophagocytic
Macrophage activation syndrome
Hemophagocytic syndrome
Hemophagocytosis
Hemophagocytic cells
Resumen
Objetivo

Describir las características demográficas y trastornos de pacientes con diagnóstico de síndrome de activación macrofágica (SAM) en el periodo comprendido entre diciembre de 2008-enero de 2014.

Métodos

Se revisaron las historias clínicas desde el diagnóstico de SAM y tras su alta hospitalaria hasta enero de 2014. Los pacientes se agruparon en 4 grupos: autoinmunes (AI), hemato-oncólogicas (HO), infecciosas (Inf) y oncológicas (Onc). Las variables fueron analizadas entre los 4 grupos y entre AI y HO.

Resultados

Trece pacientes (7 hombres, con una mediana de 54 años [32-63]) se estudiaron. Las etiologías encontradas fueron: 5 AI, 5 HO, 2 Inf y una Onc. Se encontraron células hemofagocíticas en el líquido ascítico en uno de los pacientes. Se encontró un paciente con SAM secundario a enfermedad relacionada con la IgG4.

Conclusiones

La mortalidad, el pronóstico y la evolución de la enfermedad puede verse influida por el retraso en el diagnóstico, el inicio del tratamiento y la etiología del SAM. Los pacientes con enfermedades HO presentaron peor pronóstico.

Palabras clave:
Linfohistiocitocis hemofagocítica
Síndrome de activación macrofágica
Síndrome hemofagocítico
Hemofagocitosis
Células hemofagocíticas

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