Long Term Outcomes in Paediatric Transplantation (Videos Available)

Thursday July 05, 2018 from 09:45 to 11:00

Room: Retiro Room (located next to the exhibit room)

627.4 Poorer long-term survival associated with monomorphic post-transplant lymphoproliferative disorder after solid organ transplantation in children (Video Available)

Award Winner

Fang Kuan Chiou, Singapore has been granted the TTS Young Investigator Scientific Award

Fang Kuan Chiou, Singapore

KK Women's and Children's Hospital

Abstract

Poorer Long-Term Survival Associated with Monomorphic Post-Transplant Lymphoproliferative Disorder After Solid Organ Transplantation in Children

Fang Kuan Chiou1, Khalid Sharif1, Thamara Perera1, Paolo Muiesan1, Mark Vickers2, Sue Beath1, Bruce Morland3, Darius Mirza1, Girish Gupte1.

1Liver Unit (including small bowel transplantation), Birmingham Children's Hospital, Birmingham, United Kingdom; 2Scottish National Blood Transfusion Service, Royal Infirmary, Aberdeen, United Kingdom; 3Oncology, Birmingham Children's Hospital, Birmingham, United Kingdom

Introduction: Post-transplant lymphoproliferative disorder (PTLD) is a major complication in paediatric solid organ transplantation (SOT) with poor survival rates reported at 50-80%. Less aggressive subtypes, such as early lesions and polymorphic PTLD, may respond to reduction of immunosuppression (RIS) alone. In contrast, monomorphic PTLD tends to be more advanced at presentation requiring further therapy such as rituximab (RTX), cytotoxic T-lymphocytes (CTL) and/or chemotherapy.
This study aims to review a single-centre experience focusing on the treatment outcome and long-term prognosis of monomorphic PTLD (M-PTLD) in comparison with other PTLD (O-PTLD) subtypes.
Materials and Methods: Paediatric SOT patients diagnosed with PTLD from 1989-2016 were retrospectively reviewed. PTLD was diagnosed on tissue biopsy and classified as early lesion, polymorphic, monomorphic or Hodgkin’s subtypes based on established World Health Organisation histology criteria. Treatment protocol consisted of RIS as initial treatment. RTX was introduced to the treatment protocol since year 2000. CTL was available in 1999-2004 as part of a research trial, and since 2011 as approved treatment for PTLD.
Results: Total of 37 patients were identified with median follow-up duration of 8 years (range: 1-25). Twenty-two had isolated liver (LTx), 1 had combined kidney and LTx, and 14 had intestinal transplantation with or without combined LTx. Median ages at transplant and PTLD diagnosis were 19 months (range: 6-129) and 35 months (range: 11-178) respectively. Median interval from transplant to PTLD was 6 months (range: 2–107). Most common histologic subtype was monomorphic (n=18), followed by early lesion (n=10), polymorphic (n=8) and Hodgkin’s type (n=1). There was no difference in Epstein-Barr virus (EBV) serostatus at transplant or prior history of allograft rejection or sepsis between patients with M-PTLD versus O-PTLD.  M-PTLD were more likely to present with multiple organ involvement compared to O-PTLD (67% vs 26%, p=0.014). Remission was achieved with RIS alone in 47% of patients with O-PTLD, as compared to 11% with M-PTLD (p=0.016). M-PTLD was associated with poorer overall remission rate at 6 months (67% vs 95%, p=0.029), and lower 2-year (59% vs 89%, p=0.042) and 5-year (47% vs 89%, p=0.009) survival rates compared to O-PTLD. At last follow-up, 44% of patients with M-PTLD were alive, as compared to 90% of other subtypes (p=0.003). While introduction of RTX did not appear to improve outcome, there is a significant trend towards improved survival rates in M-PTLD in association with the availability of CTL therapy. Better remission rates were achieved in CTL (86%) in M-PTLD as compared to RTX (44%).


Conclusion: M-PTLD is associated with more advanced disease and significantly poorer prognosis in paediatric SOT patients in comparison to other histologic subtypes. CTL therapy, where available, may offer a better remission rate and improve overall survival in M-PTLD.



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