Original scientific paper
Hemophagocytic Syndrome – Should We Consider it More Often?
Ivan Gornik
Vladimir Gašparović
Abstract
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.
Keywords
hemophagocytic syndrome; hemophagocytic lymphohistiocitosis; secondary
Hrčak ID:
27647
URI
Publication date:
13.12.2006.
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