Probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation: Is a biopsy still needed?

Abstract Posttransplant lymphoproliferative disorder (PTLD) is a complication of solid organ transplantation and is associated with Epstein‐Barr virus (EBV). Recently, EBV‐related PTLD was defined as probable PTLD or proven PTLD. Probable PTLD involves significant lymphadenopathy, hepatosplenomegaly...

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Main Authors: Muneyuki Matsumura, Shigehito Miyagi, Kazuaki Tokodai, Toshiaki Kashiwadate, Atsushi Fujio, Koji Miyazawa, Kengo Sasaki, Yoshikatsu Saito, Norifumi Kanai, Michiaki Unno, Takashi Kamei
Format: Article
Language:English
Published: Wiley 2022-11-01
Series:Clinical Case Reports
Subjects:
Online Access:https://doi.org/10.1002/ccr3.6454
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author Muneyuki Matsumura
Shigehito Miyagi
Kazuaki Tokodai
Toshiaki Kashiwadate
Atsushi Fujio
Koji Miyazawa
Kengo Sasaki
Yoshikatsu Saito
Norifumi Kanai
Michiaki Unno
Takashi Kamei
author_facet Muneyuki Matsumura
Shigehito Miyagi
Kazuaki Tokodai
Toshiaki Kashiwadate
Atsushi Fujio
Koji Miyazawa
Kengo Sasaki
Yoshikatsu Saito
Norifumi Kanai
Michiaki Unno
Takashi Kamei
author_sort Muneyuki Matsumura
collection DOAJ
description Abstract Posttransplant lymphoproliferative disorder (PTLD) is a complication of solid organ transplantation and is associated with Epstein‐Barr virus (EBV). Recently, EBV‐related PTLD was defined as probable PTLD or proven PTLD. Probable PTLD involves significant lymphadenopathy, hepatosplenomegaly, or other end‐organ manifestations, without a histological diagnosis, together with significant EBV DNAemia. Proven PTLD is the detection of EBV‐encoded proteins in a tissue specimen, together with symptoms and/or signs originating from the affected organ. Probable PTLD after pediatric liver transplantation has not been well documented. Therefore, here, we aimed to describe cases of five pediatric patients with probable PTLD after liver transplantation, who were successfully treated with preemptive immunosuppression reduction with or without rituximab. All five patients (age range, 1–4 years; two girls and three boys) had EBV DNAemia. Three patients developed probable PTLD within 12 months of transplantation. Further, three patients had a significantly high EBV viral load, but the other two patients with lymphadenopathy and end‐organ manifestation had a relatively low EBV viral load. Early onset pediatric PTLD with significant EBV DNAemia is almost universally EBV‐related. Biopsy was not performed in any patient due to the relative inaccessibility of the lesion and young age of the patients. If the patient’s symptoms are too mild, if excisional biopsy is too difficult to perform, or if the patient is too sick to undergo an invasive procedure, initiating preemptive treatment without a histological diagnosis could be the treatment option.
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spelling doaj.art-eaa6d794fe6a4bc783da38778129a3e32022-12-22T03:47:42ZengWileyClinical Case Reports2050-09042022-11-011011n/an/a10.1002/ccr3.6454Probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation: Is a biopsy still needed?Muneyuki Matsumura0Shigehito Miyagi1Kazuaki Tokodai2Toshiaki Kashiwadate3Atsushi Fujio4Koji Miyazawa5Kengo Sasaki6Yoshikatsu Saito7Norifumi Kanai8Michiaki Unno9Takashi Kamei10Department of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanDepartment of Surgery Tohoku University Graduate School of Medicine Sendai JapanAbstract Posttransplant lymphoproliferative disorder (PTLD) is a complication of solid organ transplantation and is associated with Epstein‐Barr virus (EBV). Recently, EBV‐related PTLD was defined as probable PTLD or proven PTLD. Probable PTLD involves significant lymphadenopathy, hepatosplenomegaly, or other end‐organ manifestations, without a histological diagnosis, together with significant EBV DNAemia. Proven PTLD is the detection of EBV‐encoded proteins in a tissue specimen, together with symptoms and/or signs originating from the affected organ. Probable PTLD after pediatric liver transplantation has not been well documented. Therefore, here, we aimed to describe cases of five pediatric patients with probable PTLD after liver transplantation, who were successfully treated with preemptive immunosuppression reduction with or without rituximab. All five patients (age range, 1–4 years; two girls and three boys) had EBV DNAemia. Three patients developed probable PTLD within 12 months of transplantation. Further, three patients had a significantly high EBV viral load, but the other two patients with lymphadenopathy and end‐organ manifestation had a relatively low EBV viral load. Early onset pediatric PTLD with significant EBV DNAemia is almost universally EBV‐related. Biopsy was not performed in any patient due to the relative inaccessibility of the lesion and young age of the patients. If the patient’s symptoms are too mild, if excisional biopsy is too difficult to perform, or if the patient is too sick to undergo an invasive procedure, initiating preemptive treatment without a histological diagnosis could be the treatment option.https://doi.org/10.1002/ccr3.6454pediatric living donor liver transplantationpreemptive therapyprobable PTLD
spellingShingle Muneyuki Matsumura
Shigehito Miyagi
Kazuaki Tokodai
Toshiaki Kashiwadate
Atsushi Fujio
Koji Miyazawa
Kengo Sasaki
Yoshikatsu Saito
Norifumi Kanai
Michiaki Unno
Takashi Kamei
Probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation: Is a biopsy still needed?
Clinical Case Reports
pediatric living donor liver transplantation
preemptive therapy
probable PTLD
title Probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation: Is a biopsy still needed?
title_full Probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation: Is a biopsy still needed?
title_fullStr Probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation: Is a biopsy still needed?
title_full_unstemmed Probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation: Is a biopsy still needed?
title_short Probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation: Is a biopsy still needed?
title_sort probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation is a biopsy still needed
topic pediatric living donor liver transplantation
preemptive therapy
probable PTLD
url https://doi.org/10.1002/ccr3.6454
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