Post‐transplant lymphoproliferative disorder after paediatric liver transplantation

Abstract

Objective The purpose of this study was to analyse the clinical and pathological characteristics, treatments and outcomes of post‐transplant lymphoproliferative disorder (PTLD) in paediatric liver transplant recipients. Method A retrospective analysis of records from nine paediatric liver transplant recipients with PTLD who were treated at our Liver Transplant Center over the period from June 2013 to August 2018. Result Of these nine patients, seven received liver transplantation in our centre and the remaining two patients at other hospitals. The overall incidence of PTLD in paediatric liver transplant recipients in our centre was 1.4% (7/485). The median onset of PTLD after liver transplantation was 11 months. Three cases were classified as infectious mononucleosis PTLD, one case was plasmacytic hyperplasia PTLD, one case was polymorphic PTLD and two cases were Burkitt lymphoma. One case showed diffuse large B‐cell lymphoma and one was classical Hodgkin lymphoma‐like PTLD. These patients presented with different clinical manifestations including fever, anaemia, diarrhoea, hypoproteinaemia, enlargement of lymph nodes, hepatosplenomegaly, jaundice, bowel obstruction and even intestinal perforation. Nine patients were positive for EBV‐DNA in serum. After diagnosis, immunosuppressants were reduced or discontinued in all cases. Eight patients received anti‐CD20 monoclonal antibody (Rituximab) therapy, four cases were treated with a combination of chemotherapy (R‐CHOP, ABVD, COPP/ABV) and one case was combined with radiotherapy. Two cases received surgical treatment due to bowel obstruction. Eight of these patients achieved a complete remission and remained healthy when assessed at the time of final follow‐up. One patient died as a result of PTLD progression. Conclusion PTLD is one of the most serious and fatal complications after liver transplantation. The definitive diagnosis requires histopathology. Treatment varies and basically includes immunosuppression reduction, anti‐CD20 antibody, surgery, radiotherapy and chemotherapy.

Coexistent Kaposi sarcoma and post-transplant lymphoproliferative disorder in the same lymph nodes after pediatric liver transplantation: A case report

“…Long-term use of immunosuppressive agents increases the risk of different diseases, including KS[ 1 , 2 ] and PTLD[ 3 - 6 ], in post-liver transplantation patients. However, to date, there are only 7 case reports[ 7 - 12 ], describing the co-occurrence of KS and lymphoid tissue lesions in the same lymph node (Table 1 ).…”

“…Post-liver transplantation patients suffer from an increased risk of developing various lesions, including Kaposi sarcoma (KS)[ 1 , 2 ] and post-transplant lymphoproliferative disorder (PTLD)[ 3 - 6 ]. KS mainly involves the skin and occasionally the lymph nodes and stomach[ 1 , 2 ], while PTLD can occur in multiple organs, including the lymph nodes and the gastrointestinal tract[ 5 ] after liver transplantation.…”

Gut and liver involvement in pediatric hematolymphoid malignancies

“…Both solid organ and hematopoietic stem-cell transplant recipients are at risk for post-transplant lymphoproliferative disorder (PTLD), a type of NHL driven mostly by Epstein-Barr virus infection. PTLD is associated highest with heart-lung, small bowel, and liver transplantations[ 86 - 88 ]. Liver and spleen involvement in PTLD is not unusual, occurring in 16% of patients over 20-years[ 85 ].…”

Not only a small liver - The pathologist’s perspective in the pediatric liver transplant setting

“…Several patterns are described, grouped by the WHO in four categories, namely (1) non-destructive PTLD, (2) polymorphic PTLD, (3) monomorphic PTLD, and (4) classical Hodgkin’s lymphoma PTLD. Although PTLD rarely affects the graft liver of PLTx patients 74-76 , more in depth discussion is beyond the aim of this manuscript. Conversely, we would like to mention and briefly illustrate the features of another EBV-related condition, that is EBV related-smooth muscle tumor (EBV-SMT).…”