28y/o AAF w/ hx of CAH, HTN, and ESRD, was on HD for 3 yrs. Pt was EBV seronegative prior to transplant (tx). She received a 4 Ag mismatch, deceased donor tx from 21/M (CMV and EBV seropositive). She received Basiliximab for induction, and started on tacrolimus (FK), mycophenolate mofetil (MMF) and prednisone for maintenance immunosuppression. Pt's nadir serum crea was 1.9 mg/dl. She had allograft biopsy 10 d post-tx due to rise in serum crea that showed Banff 2A rejection. Pt was treated with 3 doses of anti-thymocyte globulin. 6 mos later, pt was seen in clinic due to R ear pain. Amoxicillin was started for presumed diagnosis of otitis media. 3 days after starting antibiotics, pt developed trismus, fever, and bilateral periauricular swelling. CT head/neck showed enlarged parotids with lymphadenopathy. Biopsy of the L parotid showed diffuse large B cell lymphoma. Further investigations showed EBV viremia and diffuse lymphadenopathy on PET scan. Pt was started on R-CHOP therapy. FK and MMF were held. While on chemotherapy, pt developed AKI with rise in serum crea to as high as 6.9 mg/dl. Kidney biopsy showed Banff III rejection, with TMA and collapsing glomerulopathy. Pt received radiation therapy to the renal allograft and methylprednisolone IV with no improvement in renal function. US revealed no flow to the kidney tx. Pt subsequently underwent tx nephrectomy. Pathology of the kidney tx showed extensive coagulative necrosis and hemorrhage.

PTLD is the 3rd most common malignancy complicating solid organ tx but has the highest excess absolute risk when compared to general population. More than half of PTLD has extranodal presentation, and affects mucosal sites. PTLD presenting as parotitis is very rare and there are no cases yet reported in literature. Immunosuppression reduction is the cornerstone of therapy. The complete withdrawal of immunosuppression remains controversial due to risk of rejection.

CITATION INFORMATION: Pullalarevu R, Posadas Salas A, Mour G, Srinivas T, Salazar N, Mohan P. Rare Presentation of PTLD as Parotitis. Am J Transplant. 2016;16 (suppl 3).

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