P0709 TREATMENT OF INTRACTABLE ULCERATING ENTEROCOLITIS OF INFANCY BY BONE MARROW TRANSPLANTATION

Abstract

Introduction: Discontinuous Crohn’s like enterocolitis is unusual in infancy. It is usually indicative of an underlying immunodysregulation. Some such infants have so called intractable ulcerating enterocolitis of infancy (IE) in which there is characteristically marked oroanal involvement and penetrating flask like mucosal ulcers seen throughout the colon. In this condition response to conventional immunosuppressive therapy is poor, early colectomy is required and we have previously reported a high incidence (40%) of EBV driven Non-Hodgkin’s lymphoma (NHL). Methods: Patients; Two brothers (aged 8 and 5) borne of 4th cousin consangenious marriage were diagnosed with IE within the first week of life. Both had had unresponsive disease with the development of an EBV-NHL in the older sibling at age 4. They both underwent successful unrelated donor bone marrow transplantation (BMT) aged 5 and 4.8 yrs respectively. Results: These siblings have now been followed post BMT for 3 and 0.3 yrs. Both have had discontinuation of immunosuppression and no disease recurrence has been identified. Histological assessment of the older sibling reveals minor chronic inflammation in the lamina propia only. Conclusion: Infants with IE are unresponsive to conventional immunosuppressive therapy, is associated with significant morbidity and has a high risk of developing lymphoma that appears to be related to underlying immunodysregulation. Aggressive immunosuppression and acquisition of EBV infection appears to accelerate this process. Allogenic bone marrow transplant is an effective and curative therapy for this condition and is the treatment of choice.