P0648 CHRONIC T‐CELL ASSOCIATED EPSTEIN‐BARR VIRUS (EBV) INFECTION MIMICKING PEDIATRIC INFLAMMATORY BOWEL DISEASE (IBD)

Introduction: Chronic active EBV infection in which the infection is T cell, rather than B-cell driven, is increasingly being recognized as a cause of lymphoproliferative disorders in non-transplant patients. We present the case of a child with this disease who was initially diagnosed with IBD. Methods: A 9 yr old boy presented with a four year history of a debilitating chronic illness characterized by abdominal pain, diarrhea and vomiting, weight loss, anorexia, fatigue and intermittent fever. Initially, a diagnosis of colonic Crohn’s disease was entertained on the basis of patchy chronic inflammation but without granulomata. He was treated with steroids with almost complete symptom resolution but weaning proved difficult to achieve. Immunosuppressant therapy with azathioprine and methotrexate was unhelpful. He had been on almost continual therapy with prednisone in varying dosage throughout the four years preceding admission, with concomitant growth failure and poor nutrition. He developed significant hepatosplenomegaly and intra-abdominal lymphadenopathy with multiple hypodense splenic lesions, intermittent oral and skin ulceration, and high swinging fever whenever steroids were reduced. Results: Investigation was negative for angioimmunoblastic lymphadenopathy, chronic granulomatous disease, Job’s and Castleman’s syndromes, systemic mastocytosis, and metabolic storage diseases. Liver biopsy in early 2003 showed a mild portal and lobular infiltrate and EBER positivity. Laparotomy for splenic, liver and lymph node examination showed marked EBV positivity in monoclonal T cells. Gut mucosal biopsies were also EBV-positive. EBV viral loads were markedly elevated with positive EBV-VCA and EBNA. His symptoms have once again remitted on steroids and he is being considered for allogeneic bone marrow (BM) stem cell transplantation. His prognosis remains guarded. Conclusion: There are few reports of gut involvement with chronic EBV infection. Though this may initially mimic IBD, the development of a lymphoproliferative syndrome eventually enabled the diagnosis to be made. Examples of T-cell driven chronic EBV-related disorders also include hemophagocytic syndromes and T-cell/NK-cell driven lymphomas. In this context, BM stem cell transplantation may be helpful but the condition is still associated with significant morbidity and mortality.

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