Hemophagocytic Iymphohistiocytosis (HLH) is a rare and fatal immunoregulated disorders due to excessive activation of cytokins. It is characterized by hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone marrow and other tissues. Secondary HLH due to Epstein-Barr virus (EBV) infection has poor prognosis which is more progressive, more serious and has high mortality, especially during the first 2 months of onset . The authors report 2 cases of secondary HLH due to EBV infection with negative serological test of EBV and high quantity of EBV copies defined by realtime polymerase chain reaction (PCR). These patients had progressive disease, multiple organic complications, no response with combination of Dexamethasone, Etoposide, Cyclosporin A protocol and died later.
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