Treatment of intractable ulcerating enterocolitis of infancy by allogeneic bone marrow transplantation

Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association
Nikhil ThaparNeil Shah

Abstract

Intractable ulcerating enterocolitis of infancy (IE) is an uncommon, autosomal-recessive, and devastating inflammatory bowel disorder that arises as a consequence of a poorly defined underlying immunologic disorder. Infants with IE suffer from recurrent severe oro-anal disease and an enterocolitis that is unresponsive to conventional immunosuppressive therapy and requires early pancolectomy to control the severity of the disease. Despite such aggressive treatment these individuals remain at high risk of Epstein-Barr virus-driven lymphomatous proliferations, including non-Hodgkin's lymphoma. The underlying genetic basis for this disease remains undefined. This report aims to describe the use of bone marrow transplantation as a treatment for this condition. This was a case series report. We describe the successful treatment of IE by allogeneic bone marrow transplantation in 2 brothers, now aged 7 and 11 years, one of whom had developed an Epstein-Barr virus-related monomorphous B-lymphocyte lymphoproliferative disorder. This treatment has resulted in prolonged clinical remission in both boys and abrogated the need for aggressive immunosuppression. Bone marrow transplantation can be used for the treatment of intractable ulcerating...Continue Reading

References

Mar 1, 1991·Archives of Disease in Childhood·I R SandersonJ A Walker-Smith
Mar 13, 1998·Gastroenterology·S O Lopez-CuberoG B McDonald
Jun 13, 2002·Scandinavian Journal of Gastroenterology·J D SöderholmR Sjödahl
Jun 5, 2003·Transplantation·Markus DitschkowskiAhmet H Elmaagacli
Apr 30, 2005·Journal of Pediatric Gastroenterology and Nutrition·Nikhil ThaparPeter J Milla

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Citations

May 10, 2015·The Journal of Allergy and Clinical Immunology·Anna Nowak-WęgrzynSibylle Koletzko

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