# Case Report: ABO-incompatible living donor liver transplantation in a patient with associated Rosai–Dorfman–Destombes disease, first reported case

## TL;DR

A 7-year-old boy with a rare disease and chronic liver disease successfully underwent a liver transplant, a first-of-its-kind case.

## Contribution

First reported case of ABO-incompatible liver transplantation in a patient with Rosai–Dorfman–Destombes disease.

## Key findings

- ABO-incompatible LDLT was successfully performed in a patient with RDD and chronic liver disease.
- A desensitization protocol using rituximab and immunoadsorption enabled successful transplantation.
- The case highlights the potential of LDLT as a treatment option for complex RDD-related liver disease.

## Abstract

Rosai–Dorfman–Destombes disease (RDD), a rare histiocytic proliferation, is often associated with lymphadenopathy and extranodal manifestations, including involvement of the liver. We report a unique case of RDD presenting with chronic liver disease (CLD) in a 7-year-old boy, highlighting the association between these conditions. The patient underwent ABO-incompatible living donor liver transplantation (LDLT), a procedure not previously documented in the context of RDD. Successful transplantation was preceded by a desensitization protocol including rituximab and immunoadsorption, and was followed by a satisfactory postoperative course. This case underscores the need for further investigation into the relationship between RDD and CLD and the potential of LDLT as a life-saving treatment option in such complex cases.