Abstract
Epstein-Barr virus (EBV) associated lymphoproliferative disorder (LPD) after immunosuppressive therapy for aplastic anemia (AA) is extremely rare in a nontransplant setting and has not been well described. This report describes a severe AA patient in whom fatal EBV-LPD developed after being treated with rabbit antithymocyte globulins (ATG) and cyclosporine A (CsA). An 81-year-old man was diagnosed as having severe AA. He was started on CsA followed by administration of ATG for five consecutive days. One month after the start of ATG, persistent fever which was not responsive to antibiotics or antifungal agents developed and atypical lymphocytes emerged in peripheral blood. Repeated blood cultures were negative. An extremely high level of EBV virus in his peripheral blood plasma was detected by means of a quantitative real-time PCR assay. Even after the cessation of CsA, the fever persisted and the peripheral atypical lymphocytes proliferated rapidly. The patient suffered from respiratory failure, liver dysfunction, and metabolic acidosis. Rituximab was administered without success and he died.
Highlights
Acquired aplastic anemia (AA) is a rare disease characterized by pancytopenia associated with hypocellular bone marrow
Patients who underwent allogeneic Hematopoietic stem cell transplantation (HSCT) are prone to Epstein-Barr virus (EBV) reactivation and development of EBV-associated lymphoproliferative disorder (LPD) [2]
It is reported that reactivation of EBV was observed in the majority of severe AA patients treated with antithymocyte globulins (ATG) [3]
Summary
Acquired aplastic anemia (AA) is a rare disease characterized by pancytopenia associated with hypocellular bone marrow. Without treatment, it is often fatal in its severe form. Hematopoietic stem cell transplantation (HSCT) can cure severe AA and is a standard therapy for younger patients with a suitable donor. For older or HSCT ineligible severe AA patients, immunosuppressive therapy (IST) using ATG and cyclosporine A (CsA) is a standard treatment. Patients who underwent allogeneic HSCT are prone to EBV reactivation and development of EBV-associated lymphoproliferative disorder (LPD) [2]. We report a case of fatal EBV-LPD developed in an 81-year-old patient with severe AA, after being treated with rabbit ATG and CsA
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