Cytokine profile in fatal human immunodeficiency virus tuberculosis Epstein-Barr virus associated hemophagocytic syndrome.

摘要

Hemophagocytic syndrome (HPS), characterized by fever, lymphadenopathy, hepato-splenomegaly, and pancytopenia, results from the abnormal function and proliferation of macro-phages and their uncontrolled phagocytosis of various reticuloendothelial cell lines. Secondary (or "reactive") HPS is associated with infection, malignancy, or autoimmune diseases, whereas primary HPS has no identifiable cause and may be genetic. Reactive HPS has been described in association with infectious agents, but Ep-stein-Barr virus (EBV) is the most commonly associated infection, and EBV-associated HPS is almost universally fatal. At present, there is no diagnostic or treatment consensus, so HPS is generally dealt with on a case-by-case basis, depending on the associated infection(s) identified.