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

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.

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Journal
Frontiers in Transplantation
Published
2025-04-24
DOI
https://doi.org/10.3389/frtra.2025.1576301
Primary Topic
Histiocytic Disorders and Treatments
Type
article
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article

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

Vivek Vij, Kausar Makki, Vikas Saini, Piyush Srivastava et al.
Frontiers in Transplantation
Histiocytic Disorders and Treatments
article

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

Vivek Vij, Kausar Makki, Vikas Saini, Piyush Srivastava, Bhabani Sankar Sahoo
article en

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.

Frontiers in TransplantationVol. 4
Fortis Hospital (IN)
Openalex Percentile: Top 89%
Histiocytic Disorders and Treatments
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