title: Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Patients in the Black Sea Region of Turkey.
abstract: 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.
authors.affiliation: Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey.
authors.forename: Alişan
authors.initials: A
authors.lastname: Yıldıran
authors.affiliation: Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey.
authors.forename: Mehmet Halil
authors.initials: MH
authors.lastname: Çeliksoy
authors.affiliation: Leipzig University, Translational Centre for Regenerative Medicine, Leipzig, Germany.
authors.forename: Stephan
authors.initials: S
authors.lastname: Borte
authors.affiliation: Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey.
authors.forename: Şükrü Nail
authors.initials: ŞN
authors.lastname: Güner
authors.affiliation: Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey.
authors.forename: Murat
authors.initials: M
authors.lastname: Elli
authors.affiliation: Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey.
authors.forename: Tunç
authors.initials: T
authors.lastname: Fışgın
authors.affiliation: Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey.
authors.forename: Emel
authors.initials: E
authors.lastname: Özyürek
authors.affiliation: Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey.
authors.forename: Recep
authors.initials: R
authors.lastname: Sancak
authors.affiliation: Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Genetic, Samsun, Turkey.
authors.forename: Gönül
authors.initials: G
authors.lastname: Oğur
mesh_terms: D002609:Chediak-Higashi Syndrome / Q000453:epidemiology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006084:Graft Rejection / Q000453:epidemiology; D006105:Granulomatous Disease, Chronic / Q000453:epidemiology / Q000628:therapy; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology / Q000628:therapy*; D007223:Infant; D018370:Leukocyte-Adhesion Deficiency Syndrome / Q000453:epidemiology / Q000628:therapy; D051359:Lymphohistiocytosis, Hemophagocytic / Q000453:epidemiology / Q000628:therapy; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000453:epidemiology / Q000628:therapy; D016019:Survival Analysis; D014421:Turkey / Q000453:epidemiology; D014923:Wiskott-Aldrich Syndrome / Q000453:epidemiology / Q000628:therapy
keywords: Children Immunodeficiency.; Hematopoietic stem cell; Transplantation