LA MALADIE DE GAUCHER

The authors report 6 cases of type 1 Gaucher's disease, observed during 15 years between january 1980 and december 1995. These observations concern 4 babies and 2 children. Clinical presentation associated splenomegaly (6 cases), hepatomegaly (6 cases) and portal hypertension (1 case), and anemia (6 cases), pancytopenia (4 cases). The medullogram showed Gaucher's cells (6 cases). No evidence of bone lesion was found. Splenectomy was performed in 2 cases (1 baby and 1 child), in presence of hypersplenism and high volum of the spleen. The child presented a successful follow-up, meanwhile the baby presented an unsuccessful evolution.