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J Pediatr Hematol Oncol. 2018 Apr 20. doi: 10.1097/MPH.0000000000001165. [Epub ahead of print]

Hematopoietic Stem Cell Transplantation From Unrelated Donors in 2 Cases of Interleukin-10 Receptor Deficiency: Is Surgery Not a Requirement?

Author information

1
Department of Pediatric Allergy and Immunology, Faculty of Medicine, Akdeniz University.
2
Department of Pediatric Bone Marrow Transplantation Unit, Medical Park Antalya Hospital, Antalya.
3
Department of Pediatric Allergy and Immunology, Meram Faculty of Medicine.
4
Department of Pediatric Gastroenterology, Konya Training and Research Hospital, Konya, Turkey.
5
Department of Pediatric Gastroenterology, Meram Medical School, Necmettin Erbakan University.
6
Department of Medical Microbiology and Hygiene, Institute of Microbiology University Medical Center, Freiburg, Germany.
7
CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.

Abstract

Mutations in interleukin-10 and its receptors cause infantile inflammatory bowel disease (IBD), a hyperinflammatory disorder characterized by severe, treatment-refractory colitis, multiple abscesses, and enterocutaneous fistulas. Patients with infantile IBD often require several surgical interventions, including complete colectomy, and hematopoietic stem cell transplantation is currently the only known medical therapy. Traditionally, operative management has been preferred before stem cell transplantation because of the latter's increased susceptibility to procedural complications; however, surgical intervention could be delayed, and possibly reconsidered, because our 2 patients with infantile IBD demonstrated a rapid response to treatment via engraftment.

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