| Autor: |
Yıldıran A; Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey., Çeliksoy MH; Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey., Borte S; Leipzig University, Translational Centre for Regenerative Medicine, Leipzig, Germany., Güner ŞN; Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey., Elli M; Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey., Fışgın T; Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey., Özyürek E; Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey., Sancak R; Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey., Oğur G; Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Genetic, Samsun, Turkey. |
| Abstrakt: |
Hematopoietic stem cell transplantation is a promising curative therapy for many combined primary immunodeficiencies and phagocytic disorders. We retrospectively reviewed pediatric cases of patients diagnosed with primary immunodeficiencies and scheduled for hematopoietic stem cell transplantation. We identified 22 patients (median age, 6 months; age range, 1 month to 10 years) with various diagnoses who received hematopoietic stem cell transplantation. The patient diagnoses included severe combined immunodeficiency (n=11), Chediak-Higashi syndrome (n=2), leukocyte adhesion deficiency (n=2), MHC class 2 deficiency (n=2), chronic granulomatous syndrome (n=2), hemophagocytic lymphohistiocytosis (n=1), Wiskott-Aldrich syndrome (n=1), and Omenn syndrome (n=1). Of the 22 patients, 7 received human leukocyte antigen-matched related hematopoietic stem cell transplantation, 12 received haploidentical hematopoietic stem cell transplantation, and 2 received matched unrelated hematopoietic stem cell transplantation. The results showed that 5 patients had graft failure. Fourteen patients survived, yielding an overall survival rate of 67%. Screening newborn infants for primary immunodeficiency diseases may result in timely administration of hematopoietic stem cell transplantation. |
