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

https://hrcak.srce.hr/27647

Publication date:

13.12.2006.

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