Childhood Cancer

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Effects of standardized, prospective Epstein-Barr virus (EBV) monitoring on the natural history and outcomes of post-transplant lymphoproliferative disorder and EBV-associated smooth muscle tumor in pediatric solid organ transplantation patients

Institution: 
Children’s Hospital of Philadelphia
Researcher(s): 
Maanasi Gothoskar
Grant Type: 
POST Program Grants
Year Awarded: 
2024
Type of Childhood Cancer: 
Lymphoma
Project Description: 

Mentor Name: Alix Seif

Children who undergo solid organ transplantation (SOT) are at risk for Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (PTLD). PTLD is a heterogeneous disease that is often associated with the acquisition or reactivation of EBV in the context of post-transplant immunosuppression. Additionally, immunocompromised patients are at risk for rare EBV-associated tumors known as smooth muscle tumors (SMTs), and little is known about this disease entity. In 2019, we created a team of experts from multiple specialties to monitor patients for EBV after SOT in a standardized way and to review every patient who had either a positive test for or other concern of PTLD or EBV-SMT. To date, we have reviewed the clinical information on almost 400 patients with SOT. In this project, we propose a bi-directional chart review to better understand the clinical course of children who have received an SOT, with a focus on the risk factors for PTLD and SMT development. We will review our current surveillance approach in the setting of EBV viremia. We will also examine the clinical course of the disease, the variation in serum EBV titers, the role of current radiographic studies, and effectiveness of treatment regimens for children with PTLD or EBV-SMT. It is the goal of this bi-directional chart review to better understand the clinical course of children who have undergone SOT with an emphasis on PTLD development. 

Co-funded by: 
Northwestern Mutual Foundation