Linfoistiocitosi eritrofagocitica

Abstract

Erithrophagocytic lymphohistiocytosis is a rare disease characterized by exaggerated histiocytic proliferation and activation12 and multisystem involvement including visceral organs, lymph nodes, bone marrow and central nervous system4. Magnetic Resonance (MR) examination was performed in 10 patients with previously diagnosed Erithrophagocytic lymphohistiocytosis. This study was aimed at assessing MR accuracy in the identification and characterization of central nervous system lesions. MR findings show a good correlation with areas of parenchymal and meningeal lymphohistiocytic infiltration5, demonstrating diffuse white matter abnormalities in the early stages and necrotic areas with parenchymal volume loss as terminal findings4. In addition, MR allows the follow-up after chemotherapy and bone marrow transplantation2,3. Although rare, EL should be differentiated from other pediatric patchy white matter abnormalities5.