A Rare Finding at the Hepaticojejunostomy in a Postliver Transplant Patient

ABSTRACT

A 30-year-old man with a history of liver transplantation (LT) for primary sclerosing cholangitis presented with a clinical picture compatible with ascending cholangitis. The patient was started on antibiotic therapy, and a magnetic resonance imaging with cholangiopancreatography (MRCP) revealed biliary dilation. The patient then underwent double-balloon endoscopic retrograde cholangiopancreatography (DBE-ERCP). Endoscopic findings revealed an ulcerated mass obstructing the hepatico-jejunostomy (HJ), and biopsies were taken. Pathology was compatible with nondestructive post-transplant lymphoproliferative disorder, and the patient received treatment with rituximab. Eight weeks after treatment, repeat DBE-ERCP showed resolution of the mass and a patent HJ. To our knowledge, this is the first report of a diagnosis of post-transplant lymphoproliferative disorder at the HJ after LT identified with DBE-ERCP.

INTRODUCTION

Patients with liver transplantation (LT) may experience postsurgical complications, which include biliary strictures, choledocholithiasis, biliary leak, cholangitis, and rejection. Around 10%–15% of patients that undergo LT will require the creation of a Roux-en-Y hepatico-jejunostomy (HJ). Patients with this anatomical variant will not be able to undergo conventional endoscopic retrograde cholangiopancreatography (ERCP) if a biliary intervention is required. To access the biliary system through endoscopy and perform any therapeutic interventions, this group of patients will require access with balloon-assisted enteroscopy (ERCP). We describe a case of a rare pathology at the HJ in a post-LT patient diagnosed by double-balloon ERCP (DBE-ERCP).

CASE REPORT

A 30-year-old man with a history of liver transplantation (Roux-en-Y HJ) from a cadaveric donor, approximately 4 years prior for primary sclerosing cholangitis, presented with malaise, fever, and jaundice. He denied abdominal pain or other symptoms. Vital signs at triage revealed a temperature of 38.5°C (101.3°F), heart rate of 85 beats per minute, blood pressure of 125/80 mm Hg, with an oxygen saturation of 95% and respiratory rate of 20. His physical examination revealed jaundice and mild tenderness with abdominal palpation. Initial investigations showed liver enzyme elevation with aspartate aminotransferase of 195 µ/L (<55µ/L), alanine aminotransferase 271µ/L (<70µ/L), alkaline phosphatase 262µ/L (40–120µ/L), and a total bilirubin of 140 µM (<20 µM) or 8.2 mg/dL. Blood cultures taken at the time of admission were found to be positive for Escherichia coli.

The patient was noted to be on an unexpectedly aggressive regimen of chronic immunosuppressive therapy given the length of time since liver transplantation: mycophenolate mofetil 720 mg twice daily, prednisone 20 mg daily, and tacrolimus 1.5 mg daily to achieve a therapeutic range of 8–12. This was in the context of previous episodes of acute cellular rejection.

An MRI with cholangiopancreatography (MRCP) was subsequently performed. This showed bile duct dilation (8 mm) with a small filling defect (arrow) in the biliary tree (Figure 1). The patient had a good clinical response to broad-spectrum intravenous antibiotic therapy, and he then underwent a DBE-ERCP. A large ulcerated mass was identified at the HJ, through which biliary access could not be obtained as there was complete occlusion and friability at this area (Figure 2). Multiple biopsies were taken for histopathological analysis, and a percutaneous transhepatic cholangiogram was performed and an internal/external catheter was subsequently placed.

Figure 1.

Magnetic resonance cholangiopancreatography—coronal section. Bile duct dilation to 8 mm is appreciated with a small radiolucency (arrow) visualized.

Figure 2.

Initial endoscopic evaluation using double-balloon–assisted enteroscopy. A mass is appreciated in panels A and B, which made identifying the biliary orifice impossible.

Light micrographs from the lesion (Figure 3) revealed atypical appearing B cells (black arrows). Focal segments of the biopsied samples were demonstrated to be Epstein-Barr virus (EBV) RNA positive via fluorescence in situ hybridization (Figure 3); the lamina propria (Figure 3—black arrows) was shown to be intact—in keeping with a nondestructive process. Finally, Figure 3 immunohistochemical staining in keeping with a pleomorphic lymphoid population rich with B lymphocytes as evidenced by CD 20 (E) and CD 3 (F). Taken together, the findings are in keeping with early, nondestructive post-transplant lymphoproliferative disorder (PTLD). In retrospect, the EBV status of the recipient was negative, with donor status as unknown as the transplant was completed in a foreign country, and medical records were not available.

Figure 3.

Light micrographs of sampled obstructing mass at the hepaticojejunostomy. Panels A and B reveal the biopsied components were ulcerated (A) at 20×. Panel B shows a high powered field at 40× magnification. Black arrows highlighting atypical appearing B cells. Panel C shows the presence of Epstein-Barr virus RNA via fluorescence in situ hybridization. The black arrow in panel D shows a high-powered view of the lamina propria in keeping with nondestructive post-transplant lymphoproliferative disorder. Panels E and F are stained for CD 20 (E) and CD 3 (F) via immunohistochemistry, which are B-cell markers, and demonstrate a pleomorphic lymphoid population.

The patient was started on a 4-dose course of rituximab as well as EBV treatment with ganciclovir. He had an excellent clinical response, and repeat DBE 8 weeks after treatment completion revealed resolution of the ulcerating and obstructing mass (Figure 4). Biopsies confirmed absence of disease, and the percutaneous transhepatic cholangiogram drain was subsequently removed with liver enzymes improved: aminotransferase of 19µ/L (<55µ/L), alanine aminotransferase 15µ/L (<70µ/L), alkaline phosphatase 43µ/L (40-120µ/L), and a total bilirubin of 18 µM (<20 µM) or 1.05 mg/dL. During his disease course, his prednisone was decreased from 20 mg to 5 mg daily, and his tacrolimus and MMF were held entirely. Given his excellent response to rituximab, our hematologic oncology colleagues did not think that nondestructive PTLD would be FDG-avid on PET-CT and suggested it not be used as part of a surveillance plan.

Figure 4.

Endoscopic evaluation using double-balloon–assisted endoscopy of the hepatico-jejunostomy after rituximab therapy reveals the absence of the previously observed mass.

DISCUSSION

To our knowledge, this is the first report of a diagnosis of PTLD at the HJ after LT with Roux-en-Y reconstruction identified using DBE-ERCP.

PTLD is a proliferative disorder of B lymphocytes commonly seen in the post solid-organ transplant setting with EBV infection, with risk factors including degree of immunosuppression and younger transplant recipients.¹ The categorization of PTLD is based on WHO classifications.¹ The treatment of PTLD in the solid organ transplant setting varies based on classification, localization, graft function, and time from transplantation.² Treatment can include titrating immunosuppression, anti-CD20 therapy, systemic chemotherapy, or surgery if localized.

There are several reports of PTLD after LT with symptomatic presentations such as gastrointestinal bleeding, as well as painful obstructive jaundice from biliary stricturing causing cholangitis and liver abscess formation.^3,4 A recent systematic review highlighted the rarity of PTLD after LT with approximately 10,000 patients reviewed with an incidence of 2.74%. In the documented cases, treatment consisted of titration of their immunosuppressive regimen, anti-CD20 antibody treatment, EBV treatment with ganciclovir, R-CHOP treatment, or surgical resection with radiotherapy.⁵

In our case, we find that our patient was indeed negative for EBV before transplantation. Histopathologic analysis found evidence of both B- and T-cell lymphocytes in keeping with early, pleomorphic PTLD. Treatment with antivirals and anti-CD20 antibodies (rituximab) successfully eradicated this lesion.

The incidence of PTLD diagnosed endoscopically is rare,^4,6 and even more rare is the location of this patient's lesion, which required balloon-assisted enteroscopy to facilitate identification. Physicians must be aware of this condition when evaluating patients with signs of biliary obstruction after LT.

DISCLOSURES

Author contributions: J. Soleas prepared the manuscript and figures. A. Thiesen analyzed pathology images. B. Halloran edited the manuscript and provided critical article revision. S. Zepeda-Gomez performed endoscopic evaluation of the patient, prepared the manuscript, provided final approval of the article, and is the article guarantor.

Financial disclosure: Dr Halloran received a travel grant from Fuji.

Previous presentation: 2025 Canadian Digestive Disease Week, February 28–March 2, 2025, Quebec City, Canada.

Informed consent was obtained for this case report.

ABBREVIATIONS:

DBE-ERCP

double-balloon endoscopic retrograde cholangiopancreatography

HJ

hepatico-jejunostomy

LT

liver transplantation

MMF

mycophenolate mofetil

MRCP

magnetic resonance imaging with cholangiopancreatography

PTLD

post-transplant lymphoproliferative disorder

Roux-en-Y

RYEBV–Epstein Barr virus