P6016 - Sinusoidal Amyloidosis in the Setting of Acute Rejection: A Rare Finding in a Long-Term Liver Transplant Recipient

Rodolfo Singleterry, MD¹, Anish Saikumar, DO¹, Esteban Perez, DO¹, Brian Lee, DO², Lisa D. Pedicone, PhD³, Eugenia Tsai, MD³, Andres Gomez-Aldana, MD³, Jan Petrasek, MD², Fabian Rodas, MD², Carmen landaverde, MD², Eric Lawitz, MD², Fred Poordad, MD²
1Texas Institute for Graduate Medical Education and Research, San Antonio, TX; 2University of Texas Health San Antonio, San Antonio, TX; 3Texas Liver Institute, Austin, TX

Introduction: Amyloidosis is a heterogeneous group of diseases characterized by extracellular deposition of insoluble protein fibrils which can disrupt the architecture and function of vital organs. While systemic amyloidosis is well documented, its emergence following solid organ transplantation, particularly liver transplantation, is rare and poorly characterized.

Case Description/

Methods: A 54-year-old woman with a history of deceased donor liver transplant (DDLT) in 1999 secondary to Alagille syndrome, ESRD on hemodialysis, and HTN presented with hematemesis after dialysis. She had previously been adherent to her immunosuppression for 9 years before discontinuing due to socioeconomic constraints, leading to a 14-year lapse in care. Labs notable for hemoglobin: 8.7 g/dL, platelets: 130 K/mcL, INR: 1.1, albumin: 2.1 g/dL, T bilirubin: 1.4 mg/dL, ALP 251 U/L, ALT: 62 U/L, and AST: 88 U/L. Upper endoscopy revealed erosions and a visible gastric fundus vessel that was clipped. MRCP showed biliary anastomotic narrowing with upstream extrahepatic/intrahepatic biliary dilation, leading to ERCP with stent placement. Concerned for chronic graft rejection, she underwent liver biopsy showing moderate T-cell mediated rejection (rejection activity index 5/9), no advanced fibrosis, and positive Congo Red staining for sinusoidal amyloid deposition (Figures 1a and 1b). Shortly thereafter she stabilized and was discharged with outpatient follow up.

Discussion: De novo development of amyloidosis in liver transplant (LT) recipients is rare, though both acquired and hereditary forms have been reported. The DDLT occurred years earlier at an outside facility, with limited donor information but noted prior good health. Our patient had no known polyneuropathy or cardiomyopathy, typically seen in familial and wild-type transthyretin amyloidosis respectively. Her case likely reflects new-onset amyloidosis developing post-transplant. Although amyloid deposition typically does not cause acute rejection, buildup could indirectly mimic or worsen graft function. Chronic inflammation, immune dysregulation, and organ damage from rejection or infection may contribute to secondary amyloid deposition. This case underscores the complexity of post-LT care in patients with poor follow-up and highlights the importance of considering amyloidosis in atypical presentations. Moreover, it raises questions about whether long-standing allograft inflammation could contribute to amyloidogenesis in susceptible individuals.


Figure: Figure 1a. Congo red stain 20x magnification: Sinusoids are focally remarkable for amorphous pink material. Congo red stain was focally positive in sinusoidal deposits.
Figure 1b. Congo red stain, polarized 20x magnification: Congo red stain exhibiting an apple-green birefringence, indicating the presence of amyloid deposition.


Disclosures:
Rodolfo Singleterry indicated no relevant financial relationships.
Anish Saikumar indicated no relevant financial relationships.
Esteban Perez indicated no relevant financial relationships.
Brian Lee indicated no relevant financial relationships.
Lisa D. Pedicone indicated no relevant financial relationships.
Eugenia Tsai indicated no relevant financial relationships.
Andres Gomez-Aldana indicated no relevant financial relationships.
Jan Petrasek indicated no relevant financial relationships.
Fabian Rodas indicated no relevant financial relationships.
Carmen landaverde indicated no relevant financial relationships.
Eric Lawitz indicated no relevant financial relationships.
Fred Poordad indicated no relevant financial relationships.

Rodolfo Singleterry, MD¹, Anish Saikumar, DO¹, Esteban Perez, DO¹, Brian Lee, DO², Lisa D. Pedicone, PhD³, Eugenia Tsai, MD³, Andres Gomez-Aldana, MD³, Jan Petrasek, MD², Fabian Rodas, MD², Carmen landaverde, MD², Eric Lawitz, MD², Fred Poordad, MD². P6016 - Sinusoidal Amyloidosis in the Setting of Acute Rejection: A Rare Finding in a Long-Term Liver Transplant Recipient, ACG 2025 Annual Scientific Meeting Abstracts. Phoenix, AZ: American College of Gastroenterology.