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.