, Volume 89, Issue 1, pp 97-99
Date: 09 Jun 2009

Post-rituximab Burkitt transformation of PTLD: loss of CD20 expression accompanied by a switch in light-chain expression

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Dear Editor,

A 53-year-old woman, with cadaveric liver transplant for primary biliary cirrhosis 7 years ago, presented with generalized lymphadenopathy (Fig. 1a). A biopsy showed post-transplantation lymphoproliferative disease (PTLD; Fig. 1b). The lymphoma cells expressed CD20, CD79a, κ-chain, and Epstein Barr virus-encoded RNA (EBER). Her bone marrow examination was normal but a serum immunoelectrophoresis showed a monoclonal protein (IgGλ 8.2 g/l). Cyclosporin was stopped, and she was treated with R-CEOP × 6 (rituximab, cyclophosphamide, epirubicin, vincristine, and prednisolone). This was followed by acute liver rejection, requiring tacrolimus, prednisolone, and mycophenolate mofetil treatment. A remission lasted for 4 years, with undetectable circulating EBV-DNA and monoclonal protein. However, she relapsed with fever, renal shutdown, and hepatosplenomegaly (Fig. 1c). A marrow aspirate showed sheets of Burkitt-like cells (Fig. 1d), expressing CD19, CD22, CD23, EBER, and λ-chain, bu ...