Immunosuppressants
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Haemophagocytic lymphohistiocytosis triggered by Epstein-Barr virus-associated post-transplant lymphoproliferative disorder: case report A 59-year-old woman haemophagocytic lymphohistiocytosis (HLH) developed Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (PTLD) following immunosuppressive therapy with ciclosporin, mycophenolate, corticosteroids and everolimus. Following heart transplantation in 1997, the patient initially received immunosuppressive therapy with ciclosporin, mycophenolate and corticosteroids, followed by ciclosporin alone [routes and dosages not stated]. During ciclosporin therapy, her renal function gradually worsened with a serum creatinine level of 2.0 mg/dL in September 2008. To prevent terminal renal failure, everolimus 4–8 pg/µL was introduced and ciclosporin was reduced to 40–50 pg/µL. At the start of 2009, fever, recurrent infections and a generally worsening condition led to a diagnosis of EBV-associated PTLD. EBV serology was positive prior to the heart transplant, indicating EBV reactivation. Ciclosporin was further reduced. In February 2009, the patient commenced her first cycle of rituximab, leading to a significant improvement in her general condition. The number of EBV copies declined, but never fell below the detection limit. She was discharged in good condition in May 2009. Later that month, she returned with acute renal failure, recurrent urosepsis and transfusion-dependent anaemia. Laboratory findings revealed hypertriglyceridaemia, pancytopenia and high ferritin and soluble IL-2 receptor values. Abdominal ultrasound revealed splenomegaly. She was diagnosed with HLH triggered by EBV-associated PTLD, and her EBV viral load increased to more than 26 million copies/mL. Everolimus was stopped. Etoposide and dexamethasone were given for HLH, and a second rituximab course was given for EBVassociated PTLD. Neutropenic fever after chemotherapy was treated empirically. Her renal function normalised and infection and HLH parameters improved. Two weeks after discharge, she was readmitted and diagnosed with haemophagocytosis. A third cycle of rituximab was administered. Imaging revealed splenic hypermetabolism and a subsequent splenectomy was performed. Histology was consistent with HLH, and her diagnosis was EBVassociated aggressive B-cell lymphoma with ALK1 expression and plasmablastic differentiation. Her HLH parameters decreased and she was discharged in a stable condition. Two weeks later, she was admitted again with lymphoma progression and increased EBV counts. Despite antifungal treatment, she died of invasive aspergillosis. Autopsy findings included invasive lung and brain aspergillosis, PTLD and low HLH activity. Author comment: "When introducing everolimus to reduce CSA, an episode of more intense immunosuppression was generated. Although immunosuppression with everolimus may be protective in the development of PTLD due to its antiproliferative effect, this may have been counteracted by the simultaneous use of CSA, thus intensifying overall
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