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      A 5-year old male with “leukemic form” of disseminated post-transplant lymphoproliferative disorder

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          Most cited references 4

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          Post-transplant lymphoproliferative disorders occurring after renal transplantation in adults: report of 230 cases from the French Registry.

           B. Moulin,  ,  F Pessione (2006)
          Post-transplant lymphoproliferative disorders (PTLD) are a rare but serious complication after organ transplantation. A French Registry of PTLD was set up in a nationwide population of kidney transplant recipients. We prospectively enrolled all adult kidney recipients developing PTLD between January 1, 1998, and December 31, 2003. We analyzed the incidence, risk and prognostic factors of PTLD by Kaplan-Meier and Cox analyses. Totally 230 cases of PTLD were referred to the French Registry. Cumulative incidence was 1.18% after 5 years. Older age (per year, AHR = 2.19, CI = 1.22-3.94) and recipient Epstein-Barr virus seronegativity (AHR = 3.01, CI = 1.57-5.08) were associated with an increased risk of PTLD. Patients with PTLD had a reduced survival rate (61% at 5 years). Graft PTLD had the best prognosis with an 81% survival rate after 5 years. Infection with hepatitis C or B virus (HCV or HBV), late-onset PTLD, multiple sites involvement and high Ann Arbor staging were risk factors for patient death. Use of azathioprine was associated with a poorer survival rate. PTLD incidence and risk factors in French recipients are in line with the international or American PTLD series. We highlighted the role of HBV or HCV in patient mortality and described the relevant prognosis factors for patients with post-transplant lymphoproliferations.
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            Epstein-Barr virus-associated posttransplantation lymphoproliferative disorder following lung transplantation is more commonly of host origin.

            Posttransplantation lymphoproliferative disorder (PTLD) in patients who have undergone solid organ transplantation is thought to be mostly of host (ie, transplant recipient) origin, as opposed to being predominantly of donor origin, which is observed in patients who have undergone bone marrow transplantation. Donor-origin PTLDs reportedly follow a more indolent course than host-origin PTLDs. To determine the origin of PTLD and its clinical implications in patients who have undergone lung transplantation. Patients' medical records were reviewed for clinical data. We performed a molecular study to determine the origin of abnormal lymphoid cells in 4 PTLD cases identified from our autopsy files. Each case underwent restriction fragment length polymorphism analysis using polymerase chain reaction-based genotyping for CYP2D6. Epstein-Barr virus (latent membrane protein 1) immunostaining and polymerase chain reaction analysis were performed on PTLD-involved tissues. Three of 4 PTLD cases were of host origin, and the remaining case was of donor origin. Epstein-Barr virus was detected by immunohistochemical and polymerase chain reaction methods in all PTLD-involved tissues that were examined. There was no apparent difference in clinical manifestations between host-origin and donor-origin PTLD cases in our study. The PTLDs in our patients who had undergone lung transplantation were Epstein-Barr virus-positive and mostly of host origin, without any notable clinical difference from donor-origin PTLD.
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              Use of radiation therapy in posttransplant lymphoproliferative disorder (PTLD) after liver transplantation.

              Posttransplant lymphoproliferative disorder (PTLD) is a common and life-threatening complication of immunosuppression used to prevent rejection of solid organ and bone marrow transplants. There is no standardized treatment algorithm, but numerous management strategies are available. We describe a patient who developed a solitary lymphoproliferative lesion in the porta hepatis 9 months after orthotopic liver transplant. Following reduction in immunosuppression with no response, she was treated with involved field radiotherapy utilizing CT-based treatment planning. A partial radiographic response was obtained, and she has not developed disease in the engrafted liver or systemically. Based on the present case report, involved field radiotherapy seems to be a reasonable treatment option for patients with localized PTLD. Int. J. Cancer (Radiat. Oncol. Invest.) 90:104-109, 2000. Copyright 2000 Wiley-Liss, Inc.
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                Author and article information

                Affiliations
                [1 ]Department of Pathology,
                [2 ]Department of Pediatric Oncology, Georgetown University Hospital, Washington, DC, USA
                Author notes
                Correspondence: Saadiya A. Haque, Department of Pathology, Georgetown University Hospital, Washington DC, USA. E-mail: saadiyahaque@ 123456yahoo.com
                Journal
                Pediatr Rep
                PR
                PR
                Pediatric Reports
                PAGEPress Publications (Pavia, Italy )
                2036-749X
                2036-7503
                18 June 2010
                18 June 2010
                : 2
                : 1
                3094013
                21589842
                pr.2010.e6
                10.4081/pr.2010.e6
                ©Copyright S.A. Haque et al., 2010

                This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0).

                Licensee PAGEPress, Italy

                Product
                Categories
                Case Report

                Pediatrics

                ebv, lymphoproliferative, post-transplant, immunosuppression.

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