ABSTRACT
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 (AU)
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 (AU)
Subject(s)
Humans , Male , Female , Young Adult , Adult , Middle Aged , Macrophage Activation Syndrome/etiology , Autoimmune Diseases/complications , Infections/complications , Lymphohistiocytosis, Hemophagocytic/complications , Hematologic Neoplasms/complications , Retrospective Studies , Delayed DiagnosisABSTRACT
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.
Subject(s)
Autoimmune Diseases/complications , Hematologic Diseases/complications , Infections/complications , Macrophage Activation Syndrome/etiology , Neoplasms/complications , Adult , Autoimmune Diseases/diagnosis , Disease Progression , Female , Hematologic Diseases/diagnosis , Humans , Infections/diagnosis , Macrophage Activation Syndrome/diagnosis , Macrophage Activation Syndrome/mortality , Macrophage Activation Syndrome/therapy , Male , Middle Aged , Neoplasms/diagnosis , PrognosisABSTRACT
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Subject(s)
Humans , Female , Adult , Macrophage Activation Syndrome/complications , Macrophage Activation Syndrome/drug therapy , Still's Disease, Adult-Onset/complications , Still's Disease, Adult-Onset/drug therapy , Ceftriaxone/therapeutic use , Glucocorticoids/therapeutic use , Cyclosporine/therapeutic use , Still's Disease, Adult-Onset/physiopathology , Ascitic Fluid/cytology , Ascitic Fluid , Ascitic Fluid/pathologyABSTRACT
No disponible