Síndrome de Activación Macrofágica con paniculitis histiocítica citofágica en pediatría: reporte de caso
Date
Subject
macrophage activation syndrome
hemophagocytic syndrome
cytophagic histiocytic panniculitis
rare diseases
pediatrics
síndrome de activación macrofágica (SAM)
síndrome hemofagocítico
paniculitis histiocítica citofágica
enfermedades raras, pediatría
hemophagocytic syndrome
cytophagic histiocytic panniculitis
rare diseases
pediatrics
síndrome de activación macrofágica (SAM)
síndrome hemofagocítico
paniculitis histiocítica citofágica
enfermedades raras, pediatría
Language:
Journal Title
Journal ISSN
Volume Title
Publisher
Intituto Tecnológico de Santo Domingo (INTEC)
Introducción: El síndrome de activación macrofágica (SAM) es una entidad poco frecuente del sistema fagocítico-mononuclear, caracterizada por la activación benigna de macrófagos en la medula ósea, hígado, bazo y/o ganglios linfáticos asociada a alteraciones genéticas, mutaciones en el gen perforina 1 (PRF1), infecciones virales, fúngicas o bacterianas, neoplasias hematológicas, inmunodeficiencias o enfermedades autoinmunes.
Material y métodos: Al tratarse de una patología poco documentada debido a la confusión generada por los diversos nombres que ha recibido en la literatura, por su cercanía con otras patologías que cursan con un cuadro clínico muy similar, como lo es el Síndrome Hemofagocítico (SHF); presentamos el caso de un varón de 13 años de edad con síndrome de activación macrofágica, con paniculitis histiocítica citofágica de buen pronóstico, de origen esporádico.
Conclusión: La terapia con corticoesteroides en dosis baja es un punto por evaluar en esta patología, debido a la buena respuesta del tratamiento en este caso particular. Asimismo, la importancia de una intervención y manejo precoz que reduzcan considerablemente el riesgo de progresión a falla multiorgánica y la mortalidad por el síndrome.
Introduction: The Macrophage Activation Syndrome (MAS) is a rare entity of the phagocytic-mononuclear system, characterized by benign macrophage activation in the bone marrow, liver, spleen and / or lymph nodes associated genetic alterations, mutations in the perforin gene 1 (PRF1), viral, fungal or bacterial infections, hematological neoplasms, immunodeficiencies or autoimmune diseases. Material and methods: As it is a poorly documented pathology due to the confusion generated by the various names it has received in the literature due to its proximity to other pathologies that present a very similar clinical picture, such as Hemophagocytic Syndrome, we present the case of a 13-year-old male with Macrophage Activation Syndrome (MAS) with Cytogenetic Histiocytic Panniculitis of good prognosis, of sporadic origin. Conclusion: Low-dose corticosteroids therapy is a point to be evaluated in this pathology, due to the good response to the treatment in this particular case. Likewise, it is important to highlight the importance of an intervention and early management that considerably reduce the risk of progression to multi-organ failure and mortality due to the syndrome
Introduction: The Macrophage Activation Syndrome (MAS) is a rare entity of the phagocytic-mononuclear system, characterized by benign macrophage activation in the bone marrow, liver, spleen and / or lymph nodes associated genetic alterations, mutations in the perforin gene 1 (PRF1), viral, fungal or bacterial infections, hematological neoplasms, immunodeficiencies or autoimmune diseases. Material and methods: As it is a poorly documented pathology due to the confusion generated by the various names it has received in the literature due to its proximity to other pathologies that present a very similar clinical picture, such as Hemophagocytic Syndrome, we present the case of a 13-year-old male with Macrophage Activation Syndrome (MAS) with Cytogenetic Histiocytic Panniculitis of good prognosis, of sporadic origin. Conclusion: Low-dose corticosteroids therapy is a point to be evaluated in this pathology, due to the good response to the treatment in this particular case. Likewise, it is important to highlight the importance of an intervention and early management that considerably reduce the risk of progression to multi-organ failure and mortality due to the syndrome
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info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
info:eu-repo/semantics/publishedVersion
Source
Science and Health; Vol 2 No 3 (2018): Science and Health, september-december; 69-75
Ciencia y Salud; Vol. 2 Núm. 3 (2018): Ciencia y Salud, septiembre-diciembre; 69-75
2613-8824
2613-8816
10.22206/cysa.2018.v2i3
Ciencia y Salud; Vol. 2 Núm. 3 (2018): Ciencia y Salud, septiembre-diciembre; 69-75
2613-8824
2613-8816
10.22206/cysa.2018.v2i3