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Hematopoietic Stem Cell Transplantation From Unrelated Donors in 2 Cases of Interleukin-10 Receptor Deficiency

Is Surgery Not a Requirement?

Kocacik Uygun, Dilara F., MD*; Uygun, Vedat, MD; Daloğlu, Hayriye, MD; Öztürkmen, Seda, MD; Karasu, Gülsün, MD; Reisli, İsmail, MD; Sayar, Ersin, MD§; Yüksekkaya, Hasan A., MD; Glocker, Erik-Oliver, MD; Boztuğ, Kaan, MD#; Yeşilipek, Akif, MD

Journal of Pediatric Hematology/Oncology: January 2019 - Volume 41 - Issue 1 - p 64–66
doi: 10.1097/MPH.0000000000001165
Clinical and Laboratory Observations

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.

*Department of Pediatric Allergy and Immunology, Faculty of Medicine, Akdeniz University

Department of Pediatric Bone Marrow Transplantation Unit, Medical Park Antalya Hospital, Antalya

Department of Pediatric Allergy and Immunology, Meram Faculty of Medicine

Department of Pediatric Gastroenterology, Meram Medical School, Necmettin Erbakan University

§Department of Pediatric Gastroenterology, Konya Training and Research Hospital, Konya, Turkey

Department of Medical Microbiology and Hygiene, Institute of Microbiology University Medical Center, Freiburg, Germany

#CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria

The authors declare no conflict of interest.

Reprints: Vedat Uygun, MD, Department of Pediatric Bone Marrow Transplantation Unit, Medical Park Antalya Hospital, Fener Mah. Tekelioğlu Cad. No: 7 Lara, Antalya 07160, Turkey (e-mail:

Received June 2, 2017

Accepted January 25, 2018

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