Fulminant Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) with concurrent cytomegalovirus infection following matched unrelated bone marrow transplantation
Ewa Lech-Marańda, Agnieszka Wierzbowska, Agata Wrzesień-Kuś, Halina Urbańska-Ryś, Jerzy Hołowiecki, Beata Hołowiecka, Sebastian Giebel, Jacek Bartkowiak, Andrzej Bednarek, Radzisław Kordek, Tadeusz Robak
CaseRepClinPractRev 2005; 6:196-199
Background: Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) is a severe complication following allogeneic bone marrow transplantation (allo-BMT). It has been recently reported that cytomegalovirus (CMV) infection might have a contributory role in the development of
Case report: A 39-year-old female patient in first chronic phase of chronic myelogenous leukaemia (CML, Ph+) received an allo-BMT from a matched unrelated donor. The transplant was unmanipulated. On day +47 after transplantation the patient was admitted to the hospital with fever of unknown origin and icterus. Clinical, laboratory and sonographic evaluation revealed increased WBC level, increased renal and liver parameters, enlargement of lymph nodes in the hilus of liver, and hepatosplenomegaly. A bone marrow biopsy showed 70% of infiltration by lymphoplasmocytoid cells. The pp-65 CMV antigen as well as CMV-DNA and EBV-DNA by PCR analysis were detected. On day + 49 the infusion of 375/m2 rituximab was applied. Despite the treatment, the renal and liver insufficiency had progressed, and on day +51 the patient died in multiorgan failure. The
histological postmortem examination demonstrated diffuse organ infiltration by monoclonal B-cells expressing EBV-LPD. Analysis of immunoglobulin heavy chain gene (IgH) rearrangement revealed monoclonality.
Conclusions: The final diagnosis, therefore, was the fulminant EBV-LPD with the concurrent CMV reactivation. CMV and EBV infection may develop simultaneously as a result of a deficient T-cell response after allo-BMT.
Keywords: Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD), Epstein-Barr virus, Cytomegalovirus, allogeneic bone marrow transplantation