Hematopoietic stem cell transplantation from unrelated donors in children with DOCK8 deficiency
Dilara Fatma K. Uygun
Department of Pediatric Immunology, Antalya Training and Research Hospital, Antalya, Turkey
Search for more papers by this authorCorresponding Author
Vedat Uygun
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Antalya, Turkey
Correspondence
Vedat Uygun, Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Muratpaşa/Antalya, Turkey.
Email: [email protected]
Search for more papers by this authorİsmail Reisli
Meram Medical Faculty, Division of Pediatric Immunology and Allergy, Necmettin Erbakan University, Konya, Turkey
Search for more papers by this authorSevgi Keleş
Meram Medical Faculty, Division of Pediatric Immunology and Allergy, Necmettin Erbakan University, Konya, Turkey
Search for more papers by this authorAhmet Özen
Faculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, İstanbul, Turkey
Search for more papers by this authorMustafa Yılmaz
Faculty of Medicine, Division of Pediatric Allergy and Immunology, Cukurova University, Adana, Turkey
Search for more papers by this authorEsra H. Sayar
Meram Medical Faculty, Division of Pediatric Immunology and Allergy, Necmettin Erbakan University, Konya, Turkey
Search for more papers by this authorHayriye Daloğlu
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Antalya, Turkey
Search for more papers by this authorSeda I. Öztürkmen
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Antalya, Turkey
Search for more papers by this authorSuar Çakı
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Göztepe Hospital, Bahçeşehir University, İstanbul, Turkey
Search for more papers by this authorGülsün T. Karasu
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Göztepe Hospital, Bahçeşehir University, İstanbul, Turkey
Search for more papers by this authorAkif Yeşilipek
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Antalya, Turkey
Search for more papers by this authorDilara Fatma K. Uygun
Department of Pediatric Immunology, Antalya Training and Research Hospital, Antalya, Turkey
Search for more papers by this authorCorresponding Author
Vedat Uygun
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Antalya, Turkey
Correspondence
Vedat Uygun, Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Muratpaşa/Antalya, Turkey.
Email: [email protected]
Search for more papers by this authorİsmail Reisli
Meram Medical Faculty, Division of Pediatric Immunology and Allergy, Necmettin Erbakan University, Konya, Turkey
Search for more papers by this authorSevgi Keleş
Meram Medical Faculty, Division of Pediatric Immunology and Allergy, Necmettin Erbakan University, Konya, Turkey
Search for more papers by this authorAhmet Özen
Faculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, İstanbul, Turkey
Search for more papers by this authorMustafa Yılmaz
Faculty of Medicine, Division of Pediatric Allergy and Immunology, Cukurova University, Adana, Turkey
Search for more papers by this authorEsra H. Sayar
Meram Medical Faculty, Division of Pediatric Immunology and Allergy, Necmettin Erbakan University, Konya, Turkey
Search for more papers by this authorHayriye Daloğlu
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Antalya, Turkey
Search for more papers by this authorSeda I. Öztürkmen
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Antalya, Turkey
Search for more papers by this authorSuar Çakı
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Göztepe Hospital, Bahçeşehir University, İstanbul, Turkey
Search for more papers by this authorGülsün T. Karasu
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Göztepe Hospital, Bahçeşehir University, İstanbul, Turkey
Search for more papers by this authorAkif Yeşilipek
Faculty of Medicine, Department of Pediatric Bone Marrow Transplantation Unit, MedicalPark Antalya Hospital, Bahçeşehir University, Antalya, Turkey
Search for more papers by this authorAbstract
DIDS is a unique form of combined immune deficiency characterized by an unusual susceptibility to cutaneous viral infections, severe allergies with eosinophilia and elevated immunoglobulin E titers, autoimmunity, and cancer. HSCT is considered the standard of care for this deadly disease. We have retrospectively analyzed the outcome of allogeneic HSCT from unrelated donors in patients with DIDS. Data from four patients, with five transplants, are presented. All patients received transplants from unrelated donors' BM, except for one patient who received a cord blood transplant. The conditioning regimens were based on myeloablative protocols for BM derived transplants; a NM regimen was pursued for the patient who received a cord blood transplant, which resulted in graft rejection. Although recurrent pneumonia and skin infections resolved immediately after transplantation, all patients subsequently developed human herpesvirus infection, including cutaneous herpetic lesions, cytomegalovirus reactivation, and zona zoster, which could be attributed to the use of ATG. Despite the presence of serious morbidities prior to transplantation, all patients recovered successfully. DIDS can be successfully treated with allogeneic HSCT from unrelated donors following a myeloablative conditioning regimen, with a reasonable safety profile.
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