Duodenal Post-transplant Lymphoproliferative Disorder (PTLD) with Distant History of Heart Transplant: A Case Report

Abstract

A 48-year-old female with a history of heart transplant 16 years ago from Coxsackie-induced postpartum cardiomyopathy, presented with complaints of fatigue and severe diarrhea for the past month. Diarrhea was watery, non-bloody, occurring 6 times a day, unrelieved by intermittent loperamide use. She denied any sick contacts or recent travel. Her medical history consisted of renal insufficiency secondary to chronic tacrolimus toxicity, anal squamous cell cancer status post surgery with Nigro chemotherapy protocol, EBV infection, genital herpes on valacyclovir, and Kaposi sarcoma status post excision. Home medications included tacrolimus 3mg BID for transplant immunosuppression and valacyclovir 500mg QD. On exam, she demonstrated whole body anasarca, abdominal ascites, and 3+ pitting edema in her lower extremities bilaterally up to mid-back. Her labs were significant for hypoalbuminemia (2.1g/dL; normal 3.5-4.8g/dL), and hypereosinophilia (absolute eosinophil 1.4K/uL; normal 0-0.5K/uL). Urine analysis was unremarkable. Tacrolimus level was 9.9ng/mL (normal 5-20ng/mL). Infectious gastroenteritis, celiac disease, pancreatic insufficiency and inflammatory bowel disease workup was negative. The patient underwent upper endoscopy and colonoscopy with biopsies. Endoscopy showed a mildly nodular mucosa in the duodenal bulb. Colonoscopy was unremarkable. Pathology results from the duodenal bulb showed atypical lymphoid infiltrates consistent with PTLD and atypical cells that expressed CD20, CD79a, and BCL-2 and were negative for CD10. Small bowel mucosa showed eosinophilia and scattered cells tested positive for EBV. This atypical lymphoid infiltrate showed a kappa to lambda ratio of 8:1, which was consistent with her serum monoclonal gammopathy. Tacrolimus was lowered to 2mg BID due to the development of PTLD. Her symptoms markedly improved. Repeat endoscopy and colonoscopy 6 months after discharge showed remission of PTLD. The patient continued to follow-up with her physicians who recommended continuing tacrolimus 2mg BID with biweekly blood draws to ensure therapeutic levels. Valacyclovir was increased to 800mg QID. From these adjustments, albumin increased from 2.1g/dl to 3.7g/dL and she had complete resolution of anasarca and diarrhea. This case is unique because of the patient's late presentation, dramatic response to adjustments in tacrolimus and valacyclovir, and the fact that the patient developed PTLD despite normal tacrolimus levels.Figure 1Figure 2Figure 3