Risk-adapted Treatment for Severe B-Lineage Posttransplant Lymphoproliferative Disease After Solid Organ Transplantation in Children

2016 ◽  
Vol 100 (2) ◽  
pp. 437-445 ◽  
Author(s):  
Eugenia Giraldi ◽  
Massimo Provenzi ◽  
Valentino Conter ◽  
Michele Colledan ◽  
Stefania Bolognini ◽  
...  
Keyword(s):  
Organ Transplantation ◽  
Solid Organ Transplantation ◽  
Lymphoproliferative Disease ◽  
Solid Organ ◽  
Posttransplant Lymphoproliferative Disease ◽  
B Lineage

scholarly journals Epstein-Barr Virus–Positive Posttransplant Lymphoproliferative Disease After Solid Organ Transplantation

2016 ◽  
Vol 2 (1) ◽  
pp. e48 ◽  
Author(s):  
Marieke L. Nijland ◽  
Marie José Kersten ◽  
Steven T. Pals ◽  
Frederike J. Bemelman ◽  
Ineke J.M. ten Berge
Keyword(s):  
Organ Transplantation ◽  
Epstein Barr Virus ◽  
Solid Organ Transplantation ◽  
Lymphoproliferative Disease ◽  
Solid Organ ◽  
Barr Virus ◽  
Posttransplant Lymphoproliferative Disease ◽  
Epstein Barr

scholarly journals Posttransplant Lymphoproliferative Disease after Pediatric Solid Organ Transplantation

2013 ◽  
Vol 2013 ◽  
pp. 1-14 ◽  
Author(s):  
Martin Mynarek ◽  
Tilmann Schober ◽  
Uta Behrends ◽  
Britta Maecker-Kolhoff
Keyword(s):  
Organ Transplantation ◽  
Solid Organ Transplantation ◽  
Treatment Strategies ◽  
Lymphoproliferative Disease ◽  
Treatment Modalities ◽  
Solid Organ ◽  
Increased Risk ◽  
Posttransplant Lymphoproliferative Disease ◽  
New Treatment ◽  
Anti Cd20

Patients after solid organ transplantation (SOT) carry a substantially increased risk to develop malignant lymphomas. This is in part due to the immunosuppression required to maintain the function of the organ graft. Depending on the transplanted organ, up to 15% of pediatric transplant recipients acquire posttransplant lymphoproliferative disease (PTLD), and eventually 20% of those succumb to the disease. Early diagnosis of PTLD is often hampered by the unspecific symptoms and the difficult differential diagnosis, which includes atypical infections as well as graft rejection. Treatment of PTLD is limited by the high vulnerability towards antineoplastic chemotherapy in transplanted children. However, new treatment strategies and especially the introduction of the monoclonal anti-CD20 antibody rituximab have dramatically improved outcomes of PTLD. This review discusses risk factors for the development of PTLD in children, summarizes current approaches to therapy, and gives an outlook on developing new treatment modalities like targeted therapy with virus-specific T cells. Finally, monitoring strategies are evaluated.

scholarly journals Posttransplant Lymphoproliferative Disease Presenting as an Extracranial Mass

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Reuben J. Arasaratnam ◽  
Alejandro Restrepo
Keyword(s):  
Soft Tissue ◽  
Early Recognition ◽  
Solid Organ Transplantation ◽  
Organ Transplant ◽  
Antibody Therapy ◽  
B Cell Lymphoma ◽  
Lymphoproliferative Disease ◽  
Solid Organ ◽  
Tissue Mass ◽  
Posttransplant Lymphoproliferative Disease

Posttransplant lymphoproliferative disease is a serious complication following stem cell and solid organ transplantation. Early recognition of the disease is important in facilitating timely therapy and improving long-term outcomes. We report a renal transplant recipient presenting with an extracranial frontoparietal soft tissue mass that was subsequently diagnosed as a B-cell lymphoma. The patient was treated successfully with immunosuppression reduction, anti-CD20 monoclonal antibody therapy, and cytotoxic chemotherapy. Our case highlights the importance of recognizing soft tissue masses in the head and neck as a potential clinical manifestation of PTLD in solid organ transplant recipients.

Expression of CD20 but Not EBV Is Associated with Significantly Improved Overall (OS) and Event Free Survival (EFS) in Children with Post Transplant Lymphoproliferative Disease (PTLD) Following Solid Organ Transplantation (SOT).

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1787-1787
Author(s):  
Manuela A. Orjuela ◽  
Bachir Alobeid ◽  
Xinhua Liu ◽  
Aisha L. Siebert ◽  
Emily R. Kott ◽  
...  
Keyword(s):  
Organ Transplantation ◽  
Prognostic Significance ◽  
Solid Organ Transplantation ◽  
Young Patients ◽  
Lymphoproliferative Disease ◽  
Wilcoxon Test ◽  
Time Interval ◽  
Solid Organ ◽  
Post Transplant ◽  
Significant Difference

Abstract The risk of developing post transplant lymphoproliferative disease (PTLD) following solid organ transplantation (SOT) in children is in large part dependent on the specific organ transplanted and the degree of immune suppression post SOT (Webber et al Lancet 2006; Dharnidharka et al Transplantation 2001; Newell et al Transplantation 1996). However, the importance of expression of CD20 and/or Epstein-Barr virus (EBV) as prognostic factors for development and survival of pediatric PTLD after SOT in young patients are poorly understood. We previously demonstrated the safety and efficacy of cyclophosphamide, prednisone and rituximab (CPR) in CD20+ PTLD (Orjuela/Cairo, CCR 2005). We now report on our experience in children, adolescents and young adults with PTLD following SOT treated at a single institution from 1990–2008 and on the prognostic significance of expression of CD20 and EBV in this population. 45 SOT pts (28 heart, 11 liver, 6 kidney) were diagnosed with PTLD (53.3 % female) at a mean onset of 45±43 months (mo) post primary SOT (4–153). Three patients had multiple SOT. Age at diagnosis of PTLD ranged from14 to 287 mo with mean 118 (SD=77) mo. EBV and CD20 status were evaluated in all evaluable tumor sites. CD20 status was categorized as positive when all tumor sites expressed CD20 (by IHC) and negative when only some or no tumor sites expressed CD20. EBV status was categorized as positive when any tumor sites were EBV positive (by ISH), and negative when no tumor sites were EBV positive. Of 40 evaluable tumors (11 monomorphic, 19 polymorphic, 5 early lesions, 2 T-cell lymphomas, and 3 rarer types (2 HD, 1 multiple myeloma like), 32 (80%) had detectable EBV, while 28 (70%) were classified as CD20 positive. EBV expression was unrelated to age at SOT. Those patients whose PTLD expressed CD20 and/or EBV had shorter time interval between last SOT and the onset of PTLD (CD20 positive vs negative (mean±SD): 28±31 mo vs 64±44 mo, p<0.01, EBV positive vs negative: 29±24 vs 77±59 mos, p<0.02, Wilcoxon test). After controlling for the age at last SOT in the regression model for the time interval between last SOT and PTLD, expression of either CD20 or EBV was significantly associated with the shorter length of the interval. All patients had immunosuppression reduced, followed by chemo-and immunotherapy as needed. Patients with CD20 positive PTLD had a higher 5-year overall and PTLD related EFS than those patients whose PTLD was CD20 negative (92 vs 56%, p=0.02; 84 vs 29%, p<0.001, Chi-square test). The survival curve for EFS differed by CD20 status, log rank test, p<0.01 (Figure one). Organ transplant type and morphology subtype were unrelated to OS or EFS. There was no significant difference in OS or EFS based on EBV expression. In summary, expression of CD20 and/or EBV in young patients with PTLD post SOT are both important predictors of the length of time that elapses between SOT and development of PTLD. CD20 positive PTLD is associated with significantly improved 5-year OS and EFS for PTLD in young SOT patients. Patients with CD20 negative PTLD appear to have lower 5-year survival and EFS suggesting a need for alternative treatment strategies. Figure Figure

scholarly journals Multicenter Analysis of 80 Solid Organ Transplantation Recipients With Post-Transplantation Lymphoproliferative Disease: Outcomes and Prognostic Factors in the Modern Era

2010 ◽  
Vol 28 (6) ◽  
pp. 1038-1046 ◽  
Author(s):  
Andrew M. Evens ◽  
Kevin A. David ◽  
Irene Helenowski ◽  
Beverly Nelson ◽  
Dixon Kaufman ◽  
...  
Keyword(s):  
Overall Survival ◽  
Organ Transplantation ◽  
Bone Marrow Involvement ◽  
Solid Organ Transplantation ◽  
Progression Free Survival ◽  
Lymphoproliferative Disease ◽  
Solid Organ ◽  
Post Transplantation ◽  
Cart Analysis ◽  
The Impact

Purpose Adult post-transplantation lymphoproliferative disease (PTLD) has a reported 3-year overall survival (OS) of 35% to 40%. The impact of rituximab on the outcome of PTLD is not well defined. Methods We examined the clinical features and outcomes among a large cohort of solid organ transplantation (SOT) –related patients with PTLD who were recently treated at four Chicago institutions (from January 1998 to January 2008). Results Eighty patients with PTLD were identified who had a median SOT-to-PTLD time of 48 months (range, 1 to 216 months). All patients had reduction of immunosuppression as part of initial therapy, whereas 59 (74%) of 80 patients received concurrent first-line rituximab with or without chemotherapy. During 40-month median follow-up, 3-year progression-free survival (PFS) for all patients was 57%, and the 3-year overall survival (OS) rate was 62%. Patients who received rituximab-based therapy as part of initial treatment had 3-year PFS of 70% and OS 73% compared with 21% (P < .0001) and 33% (P = .0001), respectively, without rituximab. Notably, of all relapses, only 9% (4 of 34 patients) occurred beyond 12 months from PTLD diagnosis. On multivariate regression analysis, three factors were associated with progression and survival: CNS involvement (PFS, 4.70; P = .01; OS, 3.61; P = .04), bone marrow involvement (PFS, 2.95; P = .03; OS, 3.14; P = .03), and hypoalbuminemia (PFS, 2.96; P = .05; OS, 3.64; P = .04). Furthermore, a survival model by multivariate CART analysis that was based on number of adverse factors present (ie, 0, 1, ≥ 2) was formed: 3-year PFS rates were 84%, 66%, 7%, respectively, and 3-year OS rates were 93%, 68%, 11%, respectively (P < .0001). Conclusion This large, multicenter, retrospective analysis suggests significantly improved PFS and OS associated with early rituximab-based treatment in PTLD. In addition, clinical factors at diagnosis identified patients with markedly divergent outcomes.

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