Immune reconstitution in the patients with talasemia major after hematopoietic stem cell transplantation
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Dosyalar
Tarih
2020
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Turkish Society of Immunology
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Giriş: Beta?talasemi (??TM) otozomal resesif kalıtım gösteren, dünyada en yaygın hematolojik hastalıklardan birdir. Hematopoietik kök hücre transplantasyonu (HKHT) tek tedavi edici yöntem olduğundan transplantasyon sonrası hücresel immün yeniden yapılanmanın belirlenmesi başarılı klinik sonuçların anlaşılması için kritik öneme sahiptir. Bu çalışmada kök hücre transplantasyonu yapılan pediatrik yaş grubu talasemi major hastalarında lenfoid seri hücrelerin yeniden yapılanması değerlendirilmiştir. Gereçler ve Yöntemler: Kök hücre transplantasyonu yapılan 20 beta talasemili hasta çalışmaya dahil edilmiştir. Hastaların klinik ve laboratuvar bilgileri geçmişe yönelik değerlendirilmiştir. Bulgular: Transplantasyondan bir sene sonra, tüm hastalar sağ ve kan transfüzyonuna gerek duymayan bir durumdadır. Hastaların büyük çoğunluğunda CD4+ T hücre yapılanması düşüktür ve CD4+ /CD8+ hücre oranları bozulmuştur. Diğer lenfoid seri hücrelerinin yüzde ve sayıları 12 ay içinde genel olarak normal değerlere ulaştı. On yedi hastada tam donör kimerizmi görülürken yalnızca 3 hastada karışık donör kimerizmi saptandı ve yüzdeleri %55–86 arasında değişmekte idi. Sonuç: Başarılı klinik sonuçlar ve immün yeniden yapılanmaya rağmen hastaların dikkatli takip edilmesi gerekmektedir. Çünkü CD4+ T hücrelerin yapılanmasının düşük olması hastalarda ciddi enfeksiyon riski yaratabilir.
Introduction: Beta?thalassemia (??TM) is one of the most common, autosomal recessive inherited hematologic disorder in the world. Since hematopoietic stem cell transplantation (HSCT) is the only curative treatment, determination of cellular immune reconstitution is crucial for understanding of a successful clinical outcome. Here, we evaluated lymphoid reconstitution in pediatric patients with thalassemia major after stem cell transplantation. Material and Methods: The study included 20 patients with beta?thalassemia major who underwent HSCT. We assessed the clinical and laboratory information of the patients retrospectively. Results: After one year from transplantation, all patients were alive and blood transfusion independent. CD4+ T cell recovery was poor and CD4/CD8 ratio was impaired in the vast majority of the patients. Percentages and absolute counts of the other lymphoid cells generally reached the normal levels within 12 months. Seventeen patients had full donor chimerism while only 3 of 20 had low chimerism levels ranging between 55–86%. Conclusion: Although a successful clinical course and immune reconstitution were observed, the patients should be followed up carefully. Because, the poor engraftment of CD4+ T lymphocytes may lead severe infections in the patients.
Introduction: Beta?thalassemia (??TM) is one of the most common, autosomal recessive inherited hematologic disorder in the world. Since hematopoietic stem cell transplantation (HSCT) is the only curative treatment, determination of cellular immune reconstitution is crucial for understanding of a successful clinical outcome. Here, we evaluated lymphoid reconstitution in pediatric patients with thalassemia major after stem cell transplantation. Material and Methods: The study included 20 patients with beta?thalassemia major who underwent HSCT. We assessed the clinical and laboratory information of the patients retrospectively. Results: After one year from transplantation, all patients were alive and blood transfusion independent. CD4+ T cell recovery was poor and CD4/CD8 ratio was impaired in the vast majority of the patients. Percentages and absolute counts of the other lymphoid cells generally reached the normal levels within 12 months. Seventeen patients had full donor chimerism while only 3 of 20 had low chimerism levels ranging between 55–86%. Conclusion: Although a successful clinical course and immune reconstitution were observed, the patients should be followed up carefully. Because, the poor engraftment of CD4+ T lymphocytes may lead severe infections in the patients.
Açıklama
Anahtar Kelimeler
Beta-Talasemi, Kök Hücre Transplantasyonu, İmmün Yeniden Yapılanma, Beta‐Thalassemia, Immune Reconstitution, Stem Cell Transplantation
Kaynak
Turkish Journal of Immunology
WoS Q Değeri
N/A
Scopus Q Değeri
Q4
Cilt
8
Sayı
3
