Unilateral Facial Swelling as a Sign of Post-Transplant Lymphoproliferative Disorder: A Case Series

Abstract

Introduction: Post-Transplant Lymphoproliferative Disorder (PTLD) is an unfortunate but increasingly common complication in the transplant era, occurring in up to 20% of post-transplant patients. PTLD has a strong association with Epstein-Barr Virus (EBV) and commonly presents with fatigue, fever, pharyngitis, and lymphadenopathy. It rarely manifests in the head, neck and oral cavity. We present two post-transplant patients who reported unilateral facial swelling leading to a diagnosis of PTLD. Case Description: Case 1: This is a 48 year old female with past medical history of orthotopic liver transplant due to cirrhosis from biliary hypoplasia 7 months prior who presented with left-sided facial swelling and fevers. Examination showed left buccal mucosa edema and a purulent left palate lesion. Urgent surgical exploration showed a polypoid necrotic mass infiltrating the left maxilla and palate; pathology showed monomorphic B-cell lymphoproliferation. Her course was complicated by EBV and cytomegalovirus (CMV) viremia. She was treated with ganciclovir, started on rituximab for PTLD and responded well. Case 2: This is a 60 year old female with a past medical history of living unrelated donor kidney transplant due to polycystic kidney disease 9 years prior, who presented with right-sided facial swelling. She was treated with Augmentin for presumed cellulitis, however did not improve. She then presented to Otorhinolaryngology outpatient and underwent right cheek biopsy. Pathology showed diffuse large B-cell lymphoma. She was started on R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) for PTLD and responded well. Discussion: PTLD can occur in up to 20% of post-transplant patients. Risk factors for early-onset PTLD include seronegative EBV status, type of transplantation, young age at transplantation, Caucasian population, and CMV viremia. As shown, Case 1 had EBV and CMV viremia however Case 2 was EBV- and CMV-seronegative. Most commonly, PTLD presents with non-specific or gastrointestinal symptoms. In one study, PLTD in the head and neck comprised only 6% of the study population, and there have only been case reports detailing oral and gingival lesions as well as skin lesions. Although the mortality of PLTD can be as high as 60%, early detection will lead to improved survival. We postulate that post-transplant patients with unilateral facial swelling should be further evaluated for PTLD.