Sahoo Bhabani Sankar, Makki Kausar, Saini Vikas, Srivastava Piyush, Vij Vivek
Department of Liver Transplantation and Surgical Gastroenterology, Fortis Hospital, Noida, India.
Front Transplant. 2025 Apr 24;4:1576301. doi: 10.3389/frtra.2025.1576301. eCollection 2025.
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.
罗萨伊-多夫曼-德斯顿贝斯病（RDD）是一种罕见的组织细胞增生性疾病，常伴有淋巴结病和结外表现，包括肝脏受累。我们报告了一例7岁男孩患RDD并伴有慢性肝病（CLD）的独特病例，突出了这些病症之间的关联。该患者接受了ABO血型不相容的活体供肝肝移植（LDLT），这是此前在RDD背景下未记录过的手术。成功的移植手术之前采用了包括利妥昔单抗和免疫吸附的脱敏方案，术后过程令人满意。该病例强调了进一步研究RDD与CLD之间关系的必要性，以及LDLT作为此类复杂病例挽救生命治疗选择的潜力。
