A 55-year-old woman underwent living-donor
liver transplantation (LDLT). She had no history of
autoimmune diseases. Spleen was preserved.
Steroids were withdrawn at 3 months after LDLT. Epstein-Barr virus (
EBV) infection occurred at 3.5 years after LDLT. Recurrent hepatitis C virus
infection was confirmed at 4.5 years after LDLT, and pegylated
interferon was introduced. Diagnosis of EBV-positive post-transplant
lymphoproliferative disorder (PTLD) was made at 4.8 years after LDLT, and
tacrolimus (Tac) was stopped completely. Then, unconsciousness, convulsion, and cervical stiffness appeared suddenly. Electroencephalography, cerebrospinal fluid analysis, and image studies revealed normal or only nonspecific findings. The patient was in a state of exhaustion; therefore,
steroid pulse
therapy (SPT) was attempted. Surprisingly, her general condition, including consciousness disturbance, was improved markedly, and
Hashimoto's encephalopathy (HE) was suspected, based on this reaction to SPT. Elevations of
anti-thyroglobulin antibody and anti-
thyroid peroxidase antibody were confirmed. After withdrawal of Tac, and treatment with
acyclovir and
steroids, EBV-positive PTLD and HE improved, although they recurred at 5.1 years after LDLT. SPT improved only neurological symptoms.
Molecular-targeted therapy was given for recurrent PTLD, based on analysis of sampling specimens. This
therapy was effective, but
tumor lysis syndrome occurred, and the patient died at 5.3 years after LDLT.