Rare EBV-Positive Recurrent Post-Transplant Lymphoproliferative Disorder With Barely Detectable EBV Viremia: A Diagnostic Challenge

Document Type

Conference Proceeding

Publication Date

10-1-2024

Publication Title

Am J Gastroenterol

Abstract

Introduction: Post-transplant lymphoproliferative disorders (PTLD) are complications arising from post-transplantation immunosuppressive therapy. Epstein-Barr virus (EBV) viremia is often seen in PTLD, but it is not a diagnostic feature. We report a rare case of recurrent PTLD in a transplant recipient who had high EBV viremia in her first PTLD episode, however, her recurrent episode had barely detectable EBV viremia, delaying diagnosis. Case Description/Methods: A 26-year-old woman with a heart transplant (EBV donor1/recipient-) and Crohn disease presented to the emergency department with severe abdominal pain and bloody diarrhea. Her heart transplantation was 7 years previously and she was maintained on high-dose immunosuppression. Notably, she had been diagnosed with EBV-positive PTLD 4 years previously and successfully treated with rituximab. That PTLD presentation was diagnosed with substantial EBV viremia ( > 700,000 copies/mL). At this presentation, she had been treated for recurrent inflammatory bowel disease (IBD) flares over the preceding months before her symptoms escalated. Initial laboratory tests revealed high C-reactive protein, low EBV viral load below 50 IU/mL, and no lymphocyte abnormalities on peripheral smear. Computed tomography abdomen revealed severe pancolonic wall thickening and fat stranding, with multiple prominent pericolonic lymph nodes. She was started on methylprednisolone for presumed IBD flare. Due to a lack of clinical response, colonoscopy was pursued on day 3 of admission and showed inflammation with deep continuous and circumferential ulcerations from the rectum to the sigmoid, with pronounced serpiginous ulcers in the sigmoid colon. Pathology analysis (Figure 1) of colonic biopsies revealed mucosal architectural distortion, Paneth cell metaplasia, and increased lamina propria plasmacytic inflammation. Further histologic staining revealed EBV-positive plasma cell hyperplasia with no evidence of cytomegalovirus or granulomata supporting a diagnosis of recurrent early PTLD. Discussion: This case illustrates the challenge of diagnosing rare gastrointestinal recurrent PTLD. Our patient's diagnosis was delayed because her symptoms resembled an IBD flare and she had barely detectable EBV, unlike her first PTLD episode. When evaluating a transplant recipient manifesting severe gastrointestinal symptoms, we recommend maintaining a heightened suspicion for PTLD and adopting a low threshold for early endoscopy with biopsy analysis for EBV.

Volume

119

Issue

10

First Page

S1985

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