Leukoencephalopathy following combined therapy of central nervous system leukemia and lymphoma

We report a from of disseminated necrotizing leukoencephalopathy observed in five children with acute lymphoblastic leukemia or lymphoma, who received systemic chemotherapy, brain radiation, and intrathecal (IT) methotrexate, cytosine arabinoside and hydrocortisone because of meningeal tumor involvement. Three children developed a progressive neurologic disease at the end of IT therapy or shortly thereafter. The lesions consisted in discrete, apparently coalescent foci of coagulative necrosis in the white matter, with a remarkable absence of inflammatory cells, little or no tissue break-down, and striking axonal swellings. The adjacent tissue showed status spongiosus and moderate astrocytic hypertrophy. Vascular lesions were few and inconstant.