Long-Term Evaluation of Post-transplant Lymphoproliferative Disorders in Paediatric Heart Transplantation in Sao Paulo, Brazil

  • Adam ArshadEmail author
  • Estela Azeka
  • Samia Barbar
  • Raphael Marcondes
  • Adailson Siqueira
  • Luiz Benvenuti
  • Nana Miura
  • Marcelo Jatene
  • Vicente Odone Filho
Original Article


We sought to better define the demographics and characteristics of post-transplant lymphoproliferative disorders (PTLD) in a cohort of paediatric OHT patients from a developing country. Data were collected from the Heart Institute, Sao Paulo, for all paediatric OHT recipients from October 1992 to October 2018. Group differences between the PTLD and non-PTLD cohorts were assessed by Fisher exact and Mann–Whitney U tests. Kaplan–Meier curves analysed the survival in each group. Data were reviewed for 202 paediatric OHT recipients. Overall 1-, 5- and 10-year survival for the entire cohort was 76.5%, 68.3% and 62.9%; 24 patients (11.9%) developed PTLD at a median 3.1 years (IQR 0.8–9.0) after OHT. Cases were evenly spread over the follow-up period, with PTLD diagnosed in 9.8% (n = 137) of patients who were alive at 3 years, 15.3% (n = 78) of patients who were alive at 5 years and 29.3% (n = 41) of patients who were alive at 10 years. The commonest form of PTLD was diffuse large B cell lymphoma (n = 9), and most patients received rituximab with immunosuppression and chemotherapy as treatment (n = 15). We identified no increased risk in mortality amongst the PTLD vs. non-PTLD cohorts in multivariate analysis (P = 0.365). PTLD after paediatric OHT had acceptable outcomes. However, risk factors for PTLD were not identified and warrant further investigation.


Heart clinical Complications–heart clinical Paediatric transplantation 



Epstein Barr virus


Orthotopic heart transplantation


Post transplant lymphoproliferative disease



FAPESP (Fundação de Amparo a Pesquisa do Estado de Sao Paulo).

Author Contribution

Designed study EA, Data extraction EA, SB, RM, AS, LB, NM, MJ, VF, Data analysis AA, Data interpretation AA, EA, Wrote original draft AA, EA, Reviewed manuscript All authors


A public research foundation that provides grants to support research in Brazil. This funding source has had no role in the collection of data, its analysis, interpretation and the writing of this manuscript.

Compliance with Ethical Standards

Conflict of interest

The authors declare no conflicts of interest.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.College of Medical and Dental SciencesUniversity of BirminghamBirminghamUK
  2. 2.Heart Institute (InCor), University of São Paulo Medical SchoolSão PauloBrazil
  3. 3.Pediatric Oncology, University of São Paulo Medical SchoolSão PauloBrazil

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