ISSN: 1300-7777 E-ISSN: 1308-5263
Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Patients in the Black Sea Region of Turkey [Turk J Hematol]
Turk J Hematol. 2017; 34(4): 345-349 | DOI: 10.4274/tjh.2016.0477  

Hematopoietic 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

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.

Keywords: Hematopoietic stem cell, Transplantation, Children, 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

Birçok kombine primer immün yetmezlik ve fagositer bozukluk için hematopoetik kök hücre nakli küratif bir tedavidir. Bu çalışmada, primer immün yetmezlik tanısı alan ve hematopoetik kök hücre nakli yapılan hastaları retrospektif olarak inceledik. Yirmi iki hasta belirlendi. Hastaların hematopoetik kök hücre nakli sırasındaki ortanca yaşları 6 ay (minimum-maksimum: 1 ay-10 yaş) idi. Hastaların tanıları ağır kombine immün yetmezlik (n=11), Cheidak Higashi sendromu (n=2), lökosit adezyon defekti (n=2), MHC sınıf-2 eksikliği (n=2), kronik granülomatoz hastalık (n=2), hemofagositik lenfohistiyositoz (n=1), Wiskott-Aldrich sendromu (n=1) ve Omenn’s sendromu (n=1) idi. Yedi hastaya tam insan lökosit antijen uyumlu, 12 hastaya yarı uyumlu ve 2 hastaya insan lökosit antijen uyumsuz vericiden hematopoetik kök hücre nakli yapıldı. Beş hasta da graft başarısız oldu. On dört hasta hayatta kaldı ve ortalama sağkalım %67 idi. Bu hastalık için yenidoğan taramaları yapılması ile hematopoetik kök hücre transplantasyonları zamanında yapılabilir.

Anahtar Kelimeler: Hematopoetik kök hücre, Transplantasyon, Çocuk, İmmün yetmezlik


Alişan Yıldıran, Mehmet Halil Çeliksoy, Stephan Borte, Şükrü Nail Güner, Murat Elli, Tunç Fışgın, Emel Özyürek, Recep Sancak, Gönül Oğur. Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Patients in the Black Sea Region of Turkey. Turk J Hematol. 2017; 34(4): 345-349

Corresponding Author: Mehmet Halil Çeliksoy, Türkiye


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