Post‐transplantation lymphoproliferative disorders localizing to the gastrointestinal tract after liver transplantation: Report of five pediatric cases

Abstract: Post‐transplantation lymphoproliferative disorder (PTLD) is a life‐threatening complication of organ transplantation. PTLD can occur in every kind of organ transplantation. From July 1992 to July 2004, five patients were diagnosed at our transplantation center with PTLD after pediatric liver transplantation. During this period, there were 52 pediatric patients (<18 yr) receiving an orthotopic liver transplantation (OLT) at our center. All five patients had transmural gastrointestinal (GI) PTLD, which occurred mostly in the stomach and duodenum. Epstein–Barr virus (EBV) in situ was demonstrated in each case. EBV viral load was noted to be an important risk factor. Treatment included dose reduction of immunosuppressants and anti‐CD20 antibody infusion. Chemotherapy, including cyclophosphamide, doxorubicin, vincristine, and prednisolone, was given to three patients. Four patients have survived more than 10 months until now after treatment. The one who was unresponsive to chemotherapy and anti‐CD20 antibody had diffuse metastasis and died of systemic candidiasis. In our series, each PTLD involved the GI tract. The mechanism of this phenomenon is unclear, but these five cases indicate the high incidence of PTLD in pediatric solid organ transplantation.

[1]  J. Heo,et al.  Posttransplantation lymphoproliferative disorder in pediatric liver transplantation. , 2004, Transplantation proceedings.

[2]  T. The,et al.  The value of prospective monitoring of Epstein–Barr virus DNA in blood samples of pediatric liver transplant recipients , 2004, Transplant infectious disease : an official journal of the Transplantation Society.

[3]  M. Orjuela,et al.  A pilot study of chemoimmunotherapy (cyclophosphamide, prednisone, and rituximab) in patients with post-transplant lymphoproliferative disorder following solid organ transplantation. , 2003, Clinical cancer research : an official journal of the American Association for Cancer Research.

[4]  T. Berney,et al.  Successful treatment of posttransplant lymphoproliferative disease with prolonged rituximab treatment in intestinal transplant recipients , 2002, Transplantation.

[5]  G. Mazariegos,et al.  Posttransplant Lymphoproliferative Disorders in Liver Transplantation: A 20-Year Experience , 2002, Annals of surgery.

[6]  R. Sokol,et al.  Response of elevated Epstein-Barr virus DNA levels to therapeutic changes in pediatric liver transplant patients: 56-month follow up and outcome , 2002, Transplantation.

[7]  E. Sokal,et al.  Lymphoproliferation in Children After Liver Transplantation , 2002, Journal of pediatric gastroenterology and nutrition.

[8]  S. Webber,et al.  Posttransplantation lymphoproliferative disorder: manifestations in pediatric thoracic organ recipients. , 2002, Radiology.

[9]  Jaap M Middeldorp,et al.  Frequent monitoring of Epstein-Barr virus DNA load in unfractionated whole blood is essential for early detection of posttransplant lymphoproliferative disease in high-risk patients. , 2001, Blood.

[10]  B. Gridelli,et al.  Circulating EBV-DNA in the monitoring of EBV infection in pediatric liver transplant recipients. , 2001, Transplantation proceedings.

[11]  R. Porcher,et al.  Identification of prognostic factors in 61 patients with posttransplantation lymphoproliferative disorders. , 2001, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[12]  T. Gross Treatment of Epstein-Barr virus-associated posttransplant lymphoproliferative disorders. , 2001, Journal of pediatric hematology/oncology.

[13]  P. Vajro,et al.  Indications and results of chemotherapy in children with posttransplant lymphoproliferative disease after liver transplantation. , 2000, Transplantation.

[14]  G. Remuzzi,et al.  Epstein-Barr virus-negative lymphoproliferate disorders in long-term survivors after heart, kidney, and liver transplant. , 2000, Transplantation.

[15]  E. Sokal,et al.  Characteristics of Epstein-Barr virus primary infection in pediatric liver transplant recipients. , 1999, Journal of hepatology.

[16]  D. Wall,et al.  Cyclosphosphamide/prednisone for combination immunosuppression and therapy of lymphoproliferative disease. , 1999, The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation.

[17]  T. Fishbein,et al.  Prospective longitudinal analysis of quantitative Epstein-Barr virus polymerase chain reaction in pediatric liver transplant recipients. , 1999, Transplantation.

[18]  T. Morishima,et al.  Quantitative Analysis of Epstein-Barr Virus Load by Using a Real-Time PCR Assay , 1999, Journal of Clinical Microbiology.

[19]  G. Mazariegos,et al.  Serial measurement of Epstein-Barr viral load in peripheral blood in pediatric liver transplant recipients during treatment for posttransplant lymphoproliferative disease. , 1998, Transplantation.

[20]  D. Frush,et al.  Lymphoproliferative disorders: CT findings in immunocompromised children. , 1998, AJR. American journal of roentgenology.

[21]  J. Reyes,et al.  Epstein-Barr virus infections in children after transplantation of the small intestine. , 1998, The American journal of surgical pathology.

[22]  T. Greiner,et al.  Treatment of post-transplant lymphoproliferative disease (PTLD) following solid organ transplantation with low-dose chemotherapy. , 1998, Annals of oncology : official journal of the European Society for Medical Oncology.

[23]  B. Griffith,et al.  Posttransplantation lymphoproliferative disorders in pediatric thoracic organ recipients. , 1997, The Journal of pediatrics.

[24]  E. Raymond,et al.  Epstein‐Barr virus‐related localized hepatic lymphoproliferative disorders after liver transplantation , 1995, Cancer.

[25]  R. Michler,et al.  Posttransplantation lymphoproliferative disorders treated with cyclophosphamide‐doxorubicin‐vincristine‐prednisone chemotherapy , 1993, Cancer.

[26]  A. Friedman,et al.  A RECENT DECREASE IN THE TIME TO DEVELOPMENT OF MONOMORPHOUS AND POLYMORPHOUS POSTTRANSPLANT LYMPHOPROLIFERATIVE DISORDER , 1992, Transplantation.

[27]  D. Dunn,et al.  B-cell lymphoproliferative disorders in solid-organ transplant patients: detection of Epstein-Barr virus by in situ hybridization. , 1992, Human pathology.

[28]  Murphy Sb Classification, staging and end results of treatment of childhood non-Hodgkin's lymphomas: dissimilarities from lymphomas in adults. , 1980 .

[29]  S. Murphy Classification, staging and end results of treatment of childhood non-Hodgkin's lymphomas: dissimilarities from lymphomas in adults. , 1980, Seminars in oncology.