[Syndrome d'activation macrophagique: une série de 11 cas tunisiens].

BACKGROUND: Hemophagocytic syndromes are mostly associated with underlying pathology, they can reveal: immunodeficiency, infections, hemopathies, cancers and auto-immune diseases. AIM: to investigate clinical, biological features, outcome characteristics and underlying pathology of Tunisian patients with hemophagocytic syndromes. METHODS: A retrospective study of patients with hemophagocytic syndromes admitted in an internal medicine department in Tunis over the period 2009-2012. RESULTS: There were 11 patients included, 4 men and and 7 women. Mean age was 47,3years. Purpura was observed in 3 cases, hepatosplenomegaly in 6 cases and peripheral lymph nodes in 3 cases. Hemorrhagic complication was noted in 2 cases. There were an increase of inflammatory indices in all the cases. Eight patients had pancytopenia and 3 had bicytopenia. Six patients developed intravascular dissiminated coagulation. High levels of triglyceridemia was noted in 5 cases and an hyperferritinaemia in all cases. Cytological examination of bone marrow confirmed hemophagocytosis in ten cases. Hemophagocytosis was associated to infectious diseases in 6 cases, there were 2 cases of leishmaniasis, septicemia to E.Coli and staphylococcus aureus and lymph nodes tuberculosis with HIV. We also noted a case of lymphoma, myelodysplasic syndrome, rheumatoid polyarthritis, adult onset still's disease. In one patient, the origin of hemophagocytosis remained indefinite. Visceral leishmaniose were treated by Glucantime®, adult Still's disease by corticosteroids and methrotrexate®, lymphoma by chemotherapy. In lymph nodes tuberculosis, there were good evolution. Six patients died. CONCLUSION: Hemophagocytic syndrome is a rare and life-threatening disease. Pejorative prognosis requires an early therapy with etiological treatment.