SYNDROME D'ACTIVATION MACROPHAGIQUE CHEZ L ... - Toubkal

ABSTRACT:Macrophage activation syndrome (MAS) is a clinicopathological entity due toan inappropriate stimulation of macrophages. It remains a rare pathology, whichcombines non-specific clinical signs and laboratory finding. It may be primitive, orsecondary related to various diseases. This life–threatening disease have darkprognosisWe describe 12 cases of MAS, hospitalized and treated in paediatricsdepartment of Fez, during four years, since October 2005 until December 2009.The patients are between 6 months and 7 years, with 2 years and half as medium.The male to female ratio was 1.Fever was observed within 12 cases (100%), deterioration of the general state,in 12 cases, deficiency or short stature and weight in 6 cases, splenomegaly in 100%of the cases, hepatomegaly in 9 patients (75%), skin symptoms in 8 cases, oedemawithin 9 cases (75%), pleuropulmonary signs in 5 cases (42%), and 2 children wereshow haemorrhagic syndrome (16,67%).In the paraclinicals plan,the fundus Oculi was abnormal in 2 cases(papillaryHyperaemia and uveitis )pancytopenia in 7 patients (58%) and bicytopenia in 5 patients (cases 42%)(hemoglobin level is