Central nervous system involvement of graft versus host disease after allogeneic hematopoietic stem cell transplantation for adult T cell leukemia

Abstract

A 54-year-old woman was diagnosed with acute adult T-cell leukemia (ATL) in November 2015 and underwent allogeneic hematopoietic stem cell transplantation in March 2016. Cognitive impairment appeared suddenly around May 2019, and MRI of the brain showed cerebral white matter lesions. Cerebrospinal fluid examination showed no significant findings other than elevated protein. Brain biopsy showed inflammatory cells, (mainly CD8-positive T lymphocytes), infiltrating the white matter. Based on the pathological findings and the history of chronic graft versus host disease (GVHD) in the lungs and intestines, we diagnosed central nervous system involvement of GVHD (CNS-GVHD). Immunotherapy with steroids and mycophenolate mofetil resulted in improvement of the cognitive dysfunction and inflammatory findings in the spinal fluid. This case is the first report of CNS-GVHD in ATL, suggesting the importance of diagnosis by brain biopsy and the efficacy of immunotherapy.