Case Report: Post-transplant lymphoproliferative disorder after kidney transplantation in a child with schimke immuno-osseous dysplasia

TL;DR

A child with a rare immune disorder developed a rare form of lymphoproliferative disorder after a kidney transplant, highlighting the challenges of managing immunosuppression in such cases.

Contribution

This case report highlights the rare occurrence of PTLD involving the lungs in a child with SIOD and EBV seronegativity.

Findings

A child with SIOD developed EBV-associated PTLD after kidney transplantation.

The PTLD involved the lungs, an extremely rare occurrence.

Low-dose immunotherapy and stem-cell transplantation are suggested for SIOD patients.

Abstract

With the inherent T-cell immunodeficiency of Schimke immune-osseous dysplasia (SIOD), the management of immunosuppressive therapy after transplantation and life-threatening infections remains a challenge. Here, we present a case of a child with SIOD who developed early-onset Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (PTLD) after kidney transplantation. PTLD frequently involves the gastrointestinal tract and solid allografts, while this case also involved the lungs, which is extremely rare. This case underscores the importance of considering PTLD in recipients with immunodeficiency, long-term immunosuppressive therapy, and EBV seronegativity. It also suggests low-dose immunotherapy and hematopoietic stem-cell transplantation for patients with SIOD.

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