A Rare Case of T-cell Post-Transplant Lymphoproliferative Disorder (PTLD) Found Following a Diagnosis of Hemophagocytic Lymphohistiocytosis in a Patient With History of Liver Transplantation

Document Type

Conference Proceeding

Publication Date

10-1-2024

Publication Title

Am J Gastroenterol

Abstract

Introduction: Post-Transplant Lymphoproliferative Disorder (PTLD) encompasses various lymphoid proliferative disorders arising after hematopoietic or solid organ transplantation and ranging from polyclonal lesions to lymphomas. It mostly arises from B-cell origin and is linked to Ebstein-Barr Virus (EBV) in the setting of post-transplant immunosuppression. T-cell PTLD is rare, and its association with EBV is even rarer. We herein report a rare case of T-cell PTLD complicated by Hemophagocytic Lymphohistiocytosis (HLH) in a patient with history of liver transplant Case Description/Methods: A 43-year-old patient with history of alcoholic cirrhosis status post liver transplant presented with progressive bilateral lower limb weakness a year after transplant. A magnetic resonance imaging (MRI) of the spine showed cauda equina enhancements. Lumbar puncture showed inflammation and was positive for EBV. She was started on ganciclovir and bortezomib, but developed fever and pleural effusion; thoracentesis revealed exudate positive for Streptococci. Despite placement of chest tube and completion of antibiotic course, she continued to have febrile episodes with negative infectious workup except for EBV viremia. She had hemophagocytosis on bone marrow biopsy, and splenomegaly on computed tomography (CT), meeting the criteria for HLH. Her liver enzymes were elevated and CT of the liver showed bilateral multiple rounded lobular hypo-attenuating collections, the largest measuring 17 mm (Figure 1). She underwent a liver biopsy which showed EBV-positive monomorphic T-cell lymphoproliferative disorder, consistent with peripheral T-cell Lymphoma, not otherwise specified. The patient was started on CHEOP (cyclophosphamide, doxorubicin, etoposide, vincristine and prednisone) Her course was complicated with pancytopenia and recurrent attacks of fever. She eventually passed away 2 months later from septic shock Discussion: PTLD is a rare complication post solid organ transplant that is related to immune suppression. It is classically EBV related, B cell lymphoproliferation. The incidence of PTLD is higher post cardiac lung or multi organ transplant because of the higher doses of immune suppressants needed in these cases, with only 5-11% occur following liver transplant. Peripheral T-cell lymphoma is a rare PTLD subtype that is rarely associated with EBV. Vigilance post transplant is key to recognize these rare cases and guide the treatment strategy, which is usually directed towards reducing immune suppression and chemotherapy.

Volume

119

Issue

10

First Page

S2813

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