[Hemophagocytic syndrome: problems in diagnosis].
Hemophagocytic syndrome(HPS) is characterized by high-grade fever, pancytopenia, liver dysfunction and proliferation of benign histiocytes with hemophagocytosis. These clinical manifestations result from the overproduction of cytokines including interferon-gamma, interleukin-2, tumor necrosis factor-alpha by activated T cells(Th1 cells) and macrophages. HPS is divided into a hereditary/primary form and a secondary form. The latter is further classified into various entities by triggering factors and underlying diseases. The multiplicity of this syndrome makes it difficult to diagnose by a simple criteria. This paper gives a overview of HPS and describes the problems of diagnosing each sub-entity of HPS.
Tsuda
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Division of Clinical Hematology/Immunology, Kumamoto City Hospital.
D016207:Cytokines / Q000378:metabolism; D015616:Histiocytosis, Non-Langerhans-Cell / Q000145:classification / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D008223:Lymphoma / Q000150:complications; D008264:Macrophages / Q000276:immunology; D018417:Th1 Cells / Q000276:immunology; D014777:Virus Diseases / Q000150:complications
D016207:Cytokines