Non-Epstein-Barr virus-associated T-cell lymphoma following cardiac transplantation for Barth syndrome


MD Ronghe, Oncology Day Beds, Royal Hospital for Sick Children, St. Michael's Hill, Bristol BS2 8BJ, UK (Tel. +44 117 928 5520, fax. +44 117 928 5682, e-mail.


Barth syndrome is a metabolic disease characterized by infantile cardiomyopathy, neutropenia and organic aciduria. We report disease evolution in one of the first affected boys to undergo successful cardiac transplantation.

Conclusion: Although cardiac status stabilized, he developed disabling skeletal myopathy, protracted lymphopenia and-5 y after transplant-fatal Epstein Barr (EBV)-negative T-cell non-Hodgkin's lymphoma.