Post-transplant lymphoproliferative disorder (PTLD): single institutional experience of 141 patients

Rohit Bishnoi,William Birdsall Slayton,Fei Zou,Yu Wang,Aaron J Franke,William Paul Skelton,Ravneet Bajwa,Nam H Dang,Niraj M Patel

doi:10.1186/s40164-017-0087-0

Abstract

BackgroundPost-transplant lymphoproliferative disorder is a well-recognized but rare complication of hematopoietic stem cell and solid organ transplant. Due to rarity of this disease, retrospective studies from major transplant centers has been the main source to provide treatment guidelines, which are still in evolution. The sample size of this study is among one of the largest study on PTLD till date reported throughout the world.MethodsThis study was performed at University of Florida which is one of the largest transplant center in South East United States. We performed treatment and survival analysis along with univariate and multivariate analysis to identify prognostic factors.ResultsWe reviewed 141 patients diagnosed with PTLD over last 22 years with median follow-up of 2.4 years. The estimated median overall survival of the entire group was 15.0 years. Sub group analysis showed that 5-year overall survival rates of pediatric population were 88% (median not reached). For adults, median OS was 5.35 years while for elderly patients it was 1.32 years. The estimated median OS of patients with monomorphic PTLD was 9.0 years while in polymorphic PTLD was 19.3 years. Univariate analysis identified gender, age at transplant and PTLD diagnosis, performance status, IPI score, allograft type, recipient EBV status, multiple acute rejections prior to PTLD diagnosis, PTLD sub-type, extra-nodal site involvement, immunosuppressive drug regimen at diagnosis, initial treatment best response were statistically significant prognostic factors (p < 0.05). On multivariate analysis, age at PTLD diagnosis, recipient EBV status, bone marrow involvement, and initial best response were statistically significant prognostic factors (p < 0.05). Surprisingly, use of Rituximab alone as upfront therapy had poor hazard ratio in the cumulative group as well less aggressive PTLD subgroup comprising of early lesions and polymorphic PTLD.ConclusionsOur experience with treatment and analysis of outcomes does challenge current role of Rituximab use in treatment of PTLD. Currently as we define role of immunotherapy in cancer treatment, the role of acute rejections and immunosuppressant in PTLD becomes more relevant as noticed in our study. This study was also able to find new prognostic factors and also verified other known prognostic factors.

Highlights

Post-transplant lymphoproliferative disorder is a well-recognized but rare complication of hematopoietic stem cell and solid organ transplant
Mortality rates in Solid Organ Transplant (SOT)-related Post-transplant lymphoproliferative disorder (PTLD) were 50–70% and up to 70–90% in Hematopoietic Stem Cell Transplant (HSCT), recent data suggests that outcomes have been improving and comparable to SOT [10, 11]
Disease control rate (DCR) was defined as the sum of complete remission (CR), partial remission (PR), and stable disease (SD) while Overall Response Rate (ORR) was defined as the sum of CR and PR

Summary

Introduction

Post-transplant lymphoproliferative disorder is a well-recognized but rare complication of hematopoietic stem cell and solid organ transplant. Post-transplant lymphoproliferative disorder (PTLD) is a well-recognized complication of Hematopoietic Stem Cell Transplant (HSCT) and Solid Organ Transplant (SOT). It was first described in renal transplant recipients in 1968 by Doak et al [1] and the term PTLD was first introduced in 1984 by Starzl et al [2]. The reported incidence of PTLD varies in different transplant centers likely secondary to different immunosuppressive regimens, allograft types and patient population characteristics. Higher incidence of PTLD in heart, lung, intestinal and multi-organ transplants has been attributed to greater immunosuppression necessary to protect allografts in these patients [5,6,7]. Mortality rates in SOT-related PTLD were 50–70% and up to 70–90% in HSCT, recent data suggests that outcomes have been improving and comparable to SOT [10, 11]

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