Secondary macrophagic activation syndrome and Epstein Barr virus infection
Keywords:
macrophage activation syndrome, Epstein Barr virus, Juvenile idiopathic arthritisAbstract
Macrophage activation syndrome (MAS), is a clinicobiological entity, characterized by non-specific activation of monocytemacrophagic cell-line, tissular infiltration with normal activated macrophages, characterized by fever, hemophagocytosis, lymphadenopathy, hepatosplenomegaly, pancytopenia. The diagnostic criteria are: fever more than 7 days, pick >38,5°C; splenomegaly; cytopenia affecting minimum 2/3 lines unassociated to a medullar infection: Hb< 90 g/l; trombocytes < 100 x 109/l; neutrophils < 1x109/l; a high triglicerides level >2 mmol/l, a low fibrinogen < 1,5 g/l; a high feritin level > 500mcg/l; and histological criteria of medullar/hepatic, splenic or ganglionar hemophagocytosis. Macrophage activation syndrome associated with EBV infection involves infiltration of lymphoid organs with with EBV infected lymphocytes B and macrophages, with excess production of proinflammatory cytokines. We present the case of a 8 years old child, diagnosed with secondary macrophage activation syndrome.
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