P2603 - E-BelieVe It or Not!

Adam Vinall, MD¹, Grace Hopp, DO, MBA², Raina R. Flores, MD¹, Carmen Landaverde, MD³, Fabian Rodas, MD³, Jan Petrasek, MD, PhD⁴, Fred Poordad, MD³, Eric Lawitz, MD³, Lisa Pedicone, PhD⁵, Eugenia Tsai, MD^3
1University of Texas Health Science Center at San Antonio, San Antonio, TX; ²UT Health San Antonio, San Antonio, TX; ³UT Health San Antonio, Texas Liver Institute, San Antonio, TX; ⁴UT Health San Antonio, Texas Liver Institute, Jackson, MS; ⁵Texas Liver Institute, San Antonio, TX

Introduction: Post-transplant lymphoproliferative disorder (PTLD) is a rare complication of liver transplantation (LT) (1-2%) associated with Epstein-Barr virus (EBV) infection. We report an unusual case of EBV-negative PTLD that presented with lower gastrointestinal bleeding.

Case Description/Methods: A 43 year-old male three years status post deceased donor LT for hepatitis C cirrhosis with early complication of steroid-refractory, T-cell mediated rejection treated with thymoglobulin reported four days of hematochezia without associated symptoms. Laboratory results were notable for a decrease in hemoglobin from 13.9 g/dL to 10.3 g/dL over a 6 week period but with excellent graft function on mycophenolate mofetil (MMF) 250mg twice daily, tacrolimus 10mg twice daily and prednisone 5mg daily.



A colonoscopy revealed a 5.3cm partially-obstructing, ulcerative mass in the transverse colon (Figure 1a). Histology from mass biopsies demonstrated sheets of large atypical cells (Figure 1b) with CD20 (Figure 1c), CD10 and BCL-6 expression compatible with diffuse large B-cell lymphoma, confirming monomorphic B-cell PTLD. EBV PCR was negative. A PET/CT scan exhibited involvement of the small bowel and distal colon along with mesenteric, iliac and retroperitoneal lymphadenopathy. MMF was discontinued and he was treated with six cycles of cyclophosphamide, doxorubicin, prednisone, rituximab and vincristine. A follow up PET/CT and colonoscopy seven months later confirmed resolution of disease.

Discussion: PTLD is a rare but grave complication after solid organ transplantation often associated with EBV infection. In EBV(+) PTLD, immunosuppression (IS) inhibits T-cells which blocks T-cell modulation of B-cell proliferation allowing EBV-transformed B cells to multiply. Etiology of EBV(-) PTLD is not well understood but is associated with later onset and aggressive clinical behavior. Risk factors for PTLD in EBV seronegative patients are high dose IS and chronic rejection. Diagnosis is challenging due to variable and nonspecific symptoms but the gold standard is tissue biopsy. Management includes reduction in IS to restore host immunity along with administration of monoclonal antibodies or chemotherapy depending on histological features.



Our patient’s presentation of hematochezia and mild anemia illustrates the diverse and broad manifestation of PTLD. PTLD is a life-threatening complication after solid organ transplantation thus clinical vigilance and maintaining a high index of suspicion is critical to ensure early diagnosis.



Disclosures:


Adam Vinall, MD¹, Grace Hopp, DO, MBA², Raina R. Flores, MD¹, Carmen Landaverde, MD³, Fabian Rodas, MD³, Jan Petrasek, MD, PhD⁴, Fred Poordad, MD³, Eric Lawitz, MD³, Lisa Pedicone, PhD⁵, Eugenia Tsai, MD³. P2603 - E-BelieVe It or Not!, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.