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.
Gornik
Ivan
I
Division of Emergency and Intensive Care Medicine, Department of Medicine, University Hospital Rebro, Zagreb, Croatia. ivan.gornik@zg.t-com.hr
Gasparović
Vladimir
V
D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D008297:Male; D008875:Middle Aged