Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review

Merter, Mustafa; SARICI, AHMET; KUKU, IRFAN; Biçim, Soykan; Hidayet, Emine; kaya, ahmet; BERBER, Ilhami; Kaya, Emin; Uysal, Ayse; ERKURT, MEHMET ALI

Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review

Ayşe UYSAL, (Fırat Üniversitesi Tıp Fakültesi, Hematoloji Bilim Dalı, Elazığ, Türkiye)

Mehmet Ali ERKURT, (İnönü Üniversitesi Turgut Özal Tıp Merkezi, Hematoloji Bilim Dalı, Malatya, Türkiye)

Ahmet SARICI, (İnönü Üniversitesi Turgut Özal Tıp Merkezi, Hematoloji Bilim Dalı, Malatya, Türkiye)

İrfan KUKU, (İnönü Üniversitesi Turgut Özal Tıp Merkezi, Hematoloji Bilim Dalı, Malatya, Türkiye)

Soykan BİÇİM, (İnönü Üniversitesi Turgut Özal Tıp Merkezi, Hematoloji Bilim Dalı, Malatya, Türkiye)

Emine HİDAYET, (İnönü Üniversitesi Turgut Özal Tıp Merkezi, Hematoloji Bilim Dalı, Malatya, Türkiye)

Ahmet KAYA, (İnönü Üniversitesi Turgut Özal Tıp Merkezi, Hematoloji Bilim Dalı, Malatya, Türkiye)

İlhami BERBER, (İnönü Üniversitesi Turgut Özal Tıp Merkezi, Hematoloji Bilim Dalı, Malatya, Türkiye)

Emin KAYA, (İnönü Üniversitesi Turgut Özal Tıp Merkezi, Hematoloji Bilim Dalı, Malatya, Türkiye)

Mustafa MERTER (Fırat Üniversitesi Tıp Fakültesi, Hematoloji Bilim Dalı, Elazığ, Türkiye)

Fırat Tıp Dergisi

8 3

Yıl: 2023 Cilt: 28 Sayı: 1 Sayfa Aralığı: 46 - 50 Metin Dili: İngilizce İndeks Tarihi: 30-05-2023

Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review

Öz:

Objective: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the only curative treatment modality in thalassemia. Its use has been limited by age, transplant-related mortality (TRM), graft rejection, and graft versus host disease (GvHD), especially in adult patients. We aimed to present our allo-HSCT experience in adult patients with thalassemia major. Material and Method: Patients’ demographic and clinical features, donor types, resource of stem cells, conditioning regimens, GvHD prophylaxis, time to neutrophil and platelet engraftments, acute and chronic GvHD, thalassemia -free survival (TFS) and overall survival were examined. Results: The study included six patients. The median age was 21.5 (20-26) years. The median ferritin levels were 1498.4 (347.4-6992.3) pg/ml. The matched sibling donor (MSD) was used in 4 patients while matched unrelated donor (MUD) was used in 2 patients. The median time to neutrophil and platelet engraftments were 17 (15-35) and 18 (15-40) days, respectively. Acute and chronic GvHD were detected in 2 and 1 patients, respectively. The TRM was detected in 2 patients (33.3%), due to infection and acute GVHD. At a median follow-up of 28 months after transplantation, 4 (66.6%) patients were alive and TFS was achieved in 2 (33.3%) patients. Graft failure was detected in 3 (50%) patients. Conclusion: Graft rejection, TRM and GvHD limited the use of allo-HSCT, especially in adult patients. These complications were reduced by re- duced-intensity conditioning regimens and allo-HSCT should be done primarily in patients under the age of 20 years and without organ damage due to iron overload.

Anahtar Kelime:

Erişkin Talasemi Hastalarında Allojenik Hematopoetik Kök Hücre Nakli: Tek Merkez Deneyimi ve Literatür Derlemesi

Öz:

Amaç: Allojenik hematopoietik kök hücre nakli (allo-HKHN), talasemide hala tek küratif tedavi yöntemidir. Ancak yaş, transplant ilişkili mortalite, greft reddi ve greft versus host hastalığı (GvHH) nedeniyle özellikle erişkin hastalarda kullanımı sınırlıdır. Biz de erişkin talasemi majörlü hastada allo-HKHN deneyimimizi sunmayı amaçladık. Gereç ve Yöntem: Hastaların demografik ve klinik özellikleri, donör tipleri, kök hücre kaynakları, hazırlama rejimleri, GvHH profilaksisi, nötrofil ve trombosit engraftman süresi, akut ve kronik GvHH, talasemisiz sağ kalım ve toplam sağ kalım incelendi. Bulgular: Bu çalışmaya altı hasta dahil edildi. Ortanca yaş 21,5 (20-26) yıldı. Ortanca ferritin seviyeleri 1498.4 (347.4-6992.3) pg/ml idi. 4 hastada tam uyumlu kardeş donör, 2 hastada tam uyumlu akraba dışı donör kullanıldı. Nötrofil ve trombosit engraftman medyan süresi sırasıyla 17 (15-35) ve 18 (15-40) gündü. İki hastada akut ve 1 hastada kronik GvHH saptandı. Enfeksiyon ve akut GvHH nedenli olmak üzere 2 hastada (%33,3) transplant ilişkili mortalite saptandı. Nakil sonrası ortalama 28 aylık takipte 4 (%66,6) hasta hala hayatta ve 2 (%33,3) hastada talasemisiz sağ kalım sağlandı. Üç (%50) hastada greft yetmezliği saptandı. Sonuç: Greft reddi, transplant ilişkili mortalite ve GvHH, özellikle erişkin hastalarda allo-HSCT kullanımını sınırlamaktadır. Bu komplikasyonlar azaltılmış yoğunluklu hazırlama rejimleri ile azaltılmış olsa da allo-HKHN, öncelikle 20 yaşın altında ve aşırı demir yüklenmesi nedeniyle organ hasarı olmadan yapılmalıdır.

Anahtar Kelime:

Belge Türü: Makale Makale Türü: Diğer Erişim Türü: Erişime Açık

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APA	Uysal A, ERKURT M, SARICI A, KUKU I, Biçim S, Hidayet E, kaya a, BERBER I, Kaya E, Merter M (2023). Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. , 46 - 50.
Chicago	Uysal Ayse,ERKURT MEHMET ALI,SARICI AHMET,KUKU IRFAN,Biçim Soykan,Hidayet Emine,kaya ahmet,BERBER Ilhami,Kaya Emin,Merter Mustafa Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. (2023): 46 - 50.
MLA	Uysal Ayse,ERKURT MEHMET ALI,SARICI AHMET,KUKU IRFAN,Biçim Soykan,Hidayet Emine,kaya ahmet,BERBER Ilhami,Kaya Emin,Merter Mustafa Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. , 2023, ss.46 - 50.
AMA	Uysal A,ERKURT M,SARICI A,KUKU I,Biçim S,Hidayet E,kaya a,BERBER I,Kaya E,Merter M Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. . 2023; 46 - 50.
Vancouver	Uysal A,ERKURT M,SARICI A,KUKU I,Biçim S,Hidayet E,kaya a,BERBER I,Kaya E,Merter M Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. . 2023; 46 - 50.
IEEE	Uysal A,ERKURT M,SARICI A,KUKU I,Biçim S,Hidayet E,kaya a,BERBER I,Kaya E,Merter M "Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review." , ss.46 - 50, 2023.
ISNAD	Uysal, Ayse vd. "Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review". (2023), 46-50.

APA	Uysal A, ERKURT M, SARICI A, KUKU I, Biçim S, Hidayet E, kaya a, BERBER I, Kaya E, Merter M (2023). Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. Fırat Tıp Dergisi, 28(1), 46 - 50.
Chicago	Uysal Ayse,ERKURT MEHMET ALI,SARICI AHMET,KUKU IRFAN,Biçim Soykan,Hidayet Emine,kaya ahmet,BERBER Ilhami,Kaya Emin,Merter Mustafa Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. Fırat Tıp Dergisi 28, no.1 (2023): 46 - 50.
MLA	Uysal Ayse,ERKURT MEHMET ALI,SARICI AHMET,KUKU IRFAN,Biçim Soykan,Hidayet Emine,kaya ahmet,BERBER Ilhami,Kaya Emin,Merter Mustafa Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. Fırat Tıp Dergisi, vol.28, no.1, 2023, ss.46 - 50.
AMA	Uysal A,ERKURT M,SARICI A,KUKU I,Biçim S,Hidayet E,kaya a,BERBER I,Kaya E,Merter M Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. Fırat Tıp Dergisi. 2023; 28(1): 46 - 50.
Vancouver	Uysal A,ERKURT M,SARICI A,KUKU I,Biçim S,Hidayet E,kaya a,BERBER I,Kaya E,Merter M Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review. Fırat Tıp Dergisi. 2023; 28(1): 46 - 50.
IEEE	Uysal A,ERKURT M,SARICI A,KUKU I,Biçim S,Hidayet E,kaya a,BERBER I,Kaya E,Merter M "Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review." Fırat Tıp Dergisi, 28, ss.46 - 50, 2023.
ISNAD	Uysal, Ayse vd. "Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review". Fırat Tıp Dergisi 28/1 (2023), 46-50.