Erythrophagocytic Lymphohistiocytosis: MR Findings in Five Cases

Abstract

Erythrophagocytic Lymphohistiocytosis (EL) is a rare disease characterized by a haemophagocytic syndrome and multisystem involvement including visceral organs, lymph nodes, bone marrow and central nervous system. We retrospectively assessed clinical, cytological and radiological data in five patients with EL. This study was aimed at assessing MR accuracy in the identification and characterization of central nervous system lesions. Our brain MR reports, correlating with pathologic findings described in literature, show a diffuse supra- and infratentorial white matter involvement, associated with deep gray matter infiltration. The overall effect of these reports allows a correct differential diagnosis from other white matter pediatric pathologies. In the only case without neurologic symptoms, brain MR revealed a diffuse atrophy. The only patient who received a bone marrow transplantation is currently in clinical remission; brain MR control showed a substantial stabilization of the central nervous system abnormalities. MR findings show a good correlation with areas of parenchymal and meningeal lymphohistiocytic infiltration; in addition, MR shows the possible stabilization of brain parenchyma abnormalities following bone marrow transplantation.