Post-liver transplantation multicentric Castleman disease treated with valganciclovir and weaning of immunosuppression.

Abstract

Multicentric Castleman disease is a lymphoproliferative disorder which when seen in the setting of HIV/AIDS is often associated with human herpes virus 8 (HHV-8) infection. We describe the case of a HIV-negative man who developed HHV-8-associated multicentric Castleman disease 11 years after liver transplantation. The patient presented with fevers and weight loss. Physical examination revealed enlarged cervical, axillary and inguinal lymph nodes. Widespread lymphadenopathy was confirmed on computed tomography (CT) scanning. Histology of an enlarged lymph node showed a polymorphous infiltrate with mature plasma cells, plasmacytoid lymphocytes and occasional blasts within the cortex and paracortex. The diagnosis of Castleman disease was confirmed by the finding of numerous HHV-8-immunopositive cells around the regressed lymph node follicles and the detection of HHV-8 on plasma PCR. Although the conventional treatment for this condition has been combination chemotherapy, in the post-transplant context it was decided to treat the patient with valganciclovir and cessation of immunosuppression. His symptoms resolved rapidly and repeat plasma PCR done 3 months after starting treatment was negative for HHV-8. A follow-up CT scan showed a dramatic reduction in the size and amount of lymphadenopathy. After 15 months of treatment, he remains well with no evidence of graft dysfunction or rejection.