FEBRILE SYNDROME ASSOCIATED WITH MACROPHAGE ACTIVATION
Abstract:
Macrophage activation syndrome (MAS), is a clinicobiological entity, characterized by non-specific activation of monocyte-macrophagic cell-line, tissular infiltration with normal activated macrophages, by uncontrolled proliferation of histiocytes, characterized by hemophagocytosis, immune dysfunction; clinicobiologically characterized by fever, hemophagocytosis, hepatosplenomegaly, pancitopenia, hypertriglyceridemia and coagulopathy. Viral, bacterial or parasitic infections and medication play an important part in producing the disease.
Diagnosis criteria are the clinicobiological ones, such as: fever more than 7 days, pick >38,5°C, cytopenia affecting minimum 2/3 cell-lines, unassociated to a medullar infection: Hb < 9g/dl, trombocytes < 100 000/mm3, Neutrophils < 1000/mm3, a high ferritin level > 3N or over 1000 UI/l and histological criteria of medullar/hepatic, splenic or ganglionar hemophagocytosis. The treatment is based on corticotherapy, vepeside (VP 16) in monotherapy or associated to other chemotherapy, thalidomide, cyclosporine, anti-lymphocyte serum, purinic analogues, alfa-interferon, plasmapheresis, intravenous immunglobulins, antiTNF-alfa agents - etanercept.
We present the case of MI, a 82 years old male patient, diagnosed with macrophage activation syndrome.
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