ISSN: 1300-7777 E-ISSN: 1308-5263
Haematopoietic stem cell transplantation in primary immunodeficiency patients in the Black Sea Region of Turkey [Turk J Hematol]
Turk J Hematol. Ahead of Print: TJH-08218 | DOI: 10.4274/tjh.2016.0477  

Haematopoietic stem cell transplantation in primary immunodeficiency patients in the Black Sea Region of Turkey

Alişan Yıldıran1, Mehmet Halil Çeliksoy1, Stephan Borte2, Şükrü Nail Güner1, Murat Elli3, Tunç Fışgın3, Emel Özyürek3, Recep Sancak1, Gönül Oğur4
1Ondokuz Mayıs University, Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey
2Leipzig University, Translational Centre for Regenerative Medicine, Leipzig-Germany
3Ondokuz Mayıs University, Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey
4Ondokuz Mayıs University, Faculty of Medicine, Department of Pediatric Genetic, Samsun, Turkey

Objective: Haematopoietic stem cell transplantation is a promising curative therapy for many combined primary immunodeficiencies and phagocytic disorders.
Materials and Methods: We retrospectively reviewed paediatric cases that were diagnosed with primary immunodeficiencies and scheduled for haematopoietic stem cell transplantation.
Results: We identified 22 patients (median age, 6 months; age range, 1 month to 10 years) with various diagnoses who received haematopoietic stem cell transplantation. The patient diagnoses included severe combined immunodeficiency (n=11), Chediak–Higashi syndrome (n=2), leukocyte adhesion deficiency (n=2), MHC class-II 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 HLA-matched related haematopoietic stem cell transplantation, 12 received haploidentical haematopoietic stem cell transplantation, and 2 received matched unrelated haematopoietic stem cell transplantation. The results showed that 5 patients had graft failure. Fourteen patients survived, yielding an overall survival rate of 67%.
Conclusions: Screening newborn infants for primary immunodeficiency diseases may result in timely administration of haematopoietic stem cell transplantation.

Keywords: Haematopoietic stem cell, transplantation, paediatric, immunodeficiency


Primer İmmün Yetmezlikli Hastalarda Hematopoetik Kök Hücre Transplantasyonu;Türkiye'de Karadeniz Bölgesi Deneyimi

Alişan Yıldıran1, Mehmet Halil Çeliksoy1, Stephan Borte2, Şükrü Nail Güner1, Murat Elli3, Tunç Fışgın3, Emel Özyürek3, Recep Sancak1, Gönül Oğur4
1Ondokuz Mayıs University, Faculty of Medicine, Department of Pediatric Allergy and Immunology, Samsun, Turkey
2Leipzig University, Translational Centre for Regenerative Medicine, Leipzig-Germany
3Ondokuz Mayıs University, Faculty of Medicine, Department of Pediatric Hematology and Oncology, Samsun, Turkey
4Ondokuz Mayıs University, Faculty of Medicine, Department of Pediatric Genetic, Samsun, Turkey





Corresponding Author: Mehmet Halil Çeliksoy, Türkiye


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