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ISSN 0210-5187 (Electronic)

2006, Number 4

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Med Cutan Iber Lat Am 2006; 34 (4)

Macrophage activation syndrome

Kouris E, Giansante E

Language: Spanish
References: 34
Page: 145-154
PDF size: 267.32 Kb.


ABSTRACT

The macrophage activation syndrome (MAS) is a rare and potencially fatal disorder, secondary to the activation of the system phagocytic mononuclear that is characterized by the proliferation uncontrolled of the histiocytes with hemophagocytosis phenomenon. Is a disorder of the immune regulation characterized by fever, hemophagocytosis, hepatoesplenomegaly, pancytopenia, hipertriglyceridemy and coagulopatic. The etiology of the MAS is unknown but factors such desencadenantes are described as viral infections and you drug. The excessive activation and proliferation of the well differentiated macrophages and of the T cells it induces a high amount de pro-infl ammatory cytokines. It has been postulated that it exists an important decrease of the cells natural killer and a functional defect in the perforin. The clinical course of the MAS is variable. Clinically it is similar to the hemophagocytic lynphohistiocytosis reactive associated to virus and the family form. The most frequent cutaneous manifestations are the panniculitis and the purple; the cytophagic histiocytic panniculitis (CHP) it is the cutaneous manifestation it specifi es. The CHP can have a benign course and a more aggressive form that it corresponds to the subcutaneous panniculitis T-cells lymphoma. The earlier diagnosis of this pathology is critical in prognosis. It has been described that the measurement of the serum ferritin level may assist in the diagnosis and may be a useful indicador of disease activity. At the moment, the election treatment is the intravenous corticosteroids. The cyclosporin use and antitumoral necrosis factor alpha agents as etarnecept have been evaluated, with good results.


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