Hemophagocytic syndrome-Should we consider it more often? (CROSBI ID 132969)
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Gornik, Ivan ; Gašparović, Vladimir
engleski
Hemophagocytic syndrome-Should we consider it more often?
Hemophagocytic syndrome (HPS) is a rare condition characterized by overactive histiocytes, hepatosplenomegaly, fever and cytopenia, with two major types: familial, autosomal recessive genetic disease and acquired that can occur during systemic infections, immunodeficiency or malignancy. Inappropriate activation of macrophages by cytokines is the major mechanism of the disease. We report a case of an adult patient with HPS. After thorough clinical investigation, we have not been able to establish the underlying disease, and corticosteroids therapy was initiated empirically. After 8 months follow-up the patient is well with normal laboratory findings.
hemophagoytic syndrome; hemophagoytic lymphohistiocitosis
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