Case 2
A 17-year-old male with a past medical history of b cell deficiency and inflammatory colitis was admitted to our facility after failed treatment for presumed pneumonia and found to have severe dilated cardiomyopathy (idiopathic vs autoimmune) eventually necessitating heart transplant. Pre-transplant serologies were EBV donor positive/recipient positive. Peri-operatively, he received two doses of ATG and corticosteroids. Initial immunosuppression consisted of tacrolimus, MMF, prednisone, and sulfasalazine (for colitis), which were eventually weaned. Approximately three years following transplant, he was admitted to the hospital for evaluation of weight loss, headache, anemia and diarrhea. On admission, EBV DNA was elevated at 10,058 copies/mL; previously negative the year prior. EBV levels continued to increase during admission to a peak of 161, 476 copies/mL. PET showed FDG avid lesions in multiple areas including the liver (six cm lesion in the caudate lobe), colon, femur, thymus, and lungs. MRI brain and PET demonstrated a mass in the right meckel’s cave suspicious for PTLD. He underwent colonoscopy which revealed lesions in the gastric antrum (not visualized on PET) and ascending colon concerning for PTLD. Biopsies of the gastric antrum and colon were negative for immunophenotypic evidence of lymphoproliferative disorder. However, subsequent liver biopsy confirmed EBV SMT (Fig 2). He was started on Imatanib which was discontinued shortly thereafter due to concerns of severe drug rash. Surgical resection of the liver mass was felt to have limited efficacy given the multiple foci of disease and was not pursued. Following Imatinib, in an effort to promote anti-tumor immune response due to advanced disease, all immunosuppression was discontinued approximately three weeks following diagnosis. At approximately 2 months following EBV SMT diagnosis, he developed acute respiratory distress and multi-organ failure that led to death secondary to progressive malignancy.