TY - JOUR AU - Fisman, David N. T1 - Hemophagocytic Syndromes and Infection T2 - Emerging Infectious Disease journal PY - 2000 VL - 6 IS - 6 SP - 601 SN - 1080-6059 AB - Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone marrow and other tissues. HLH may be diagnosed in association with malignant, genetic, or autoimmune diseases but is also prominently linked with Epstein-Barr (EBV) virus infection. Hyperproduction of cytokines, including interferon-γ and tumor necrosis factor-α, by EBV-infected T lymphocytes may play a role in the pathogenesis of HLH. EBV-associated HLH may mimic T-cell lymphoma and is treated with cytotoxic chemotherapy, while hemophagocytic syndromes associated with nonviral pathogens often respond to treatment of the underlying infection. KW - Hemophagocytic lymphohistiocytosis KW - HLH KW - hemophagocytosis KW - Epstein-Barr KW - EBV virus KW - United States DO - 10.3201/eid0606.000608 UR - https://wwwnc.cdc.gov/eid/article/6/6/00-0608_article ER - End of Reference