. 2021; 38(3): 195-203 | DOI: 10.4274/tjh.galenos.2021.2021.0105 | |||
Hematopoietic Stem Cell Transplantation for Patients with Paroxysmal Nocturnal Hemoglobinuria with or without Aplastic Anemia: A Multicenter Turkish ExperienceFergün Yılmaz1, Nur Soyer2, Güldane Cengiz Seval3, Sinem Civriz Bozdağ4, Pervin Topcuoğlu3, Ali Ünal5, Leylagül Kaynar6, Gökhan Özgür7, Gülsan Türköz Sucak8, Hakan Göker9, Mustafa Velet9, Hakan Özdoğu10, Mehmet Yılmaz11, Emin Kaya12, Ozan Salim13, Burak Deveci14, İhsan Karadoğan14, Güray Saydam2, Fahri Şahin2, Filiz Vural21Marmara University School of Medicine, Department of Hematology, Istanbul, Turkey;PESG, PNH and Education Study Group2Ege University School of Medicine, Department of Hematology, Izmir, Turkey;PESG, PNH and Education Study Group 3Ankara University School of Medicine, Department of Hematology, Ankara, Turkey 4Ankara University School of Medicine, Department of Hematology, Ankara, Turkey;PESG, PNH and Education Study Group 5Erciyes University School of Medicine, Department of Hematology, Ankara, Turkey;PESG, PNH and Education Study Group 6Erciyes University School of Medicine, Department of Hematology, Ankara, Turkey 7Medical Park, Bahcesehir Hospital, Hematology and Transplantation Clinic, Istanbul, Turkey; PESG, PNH and Education Study Group 8Medical Park, Bahcesehir Hospital, Hematology and Transplantation Clinic, Istanbul, Turkey 9Hacettepe University School of Medicine, Department of Hematology, Ankara, Turkey 10Baskent University School of Medicine, Department of Hematology, Adana Bone Marrow Transplantation Center, Adana, Turkey 11SANKO University School of Medicine, Department of Hematology, Gaziantep, Turkey;PESG, PNH and Education Study Group 12İnönü University School of Medicine, Department of Hematology, Malatya, Turkey;PESG, PNH and Education Study Group 13Akdeniz University, School of Medicine, Department of Hematology, Antalya, Turkey; PESG, PNH and Education Study Group 14Medstar Antalya Hospital Bone Marrow Transplantation Center / Istanbul Gelisim University, Department of Hematology, Antalya, Turkey Objective: Although inhibition of the complement system at different steps is a promising therapy modality in patients with paroxysmal nocturnal hemoglobinuria (PNH), allogeneic hematopoietic stem cell transplantation (HCT) is still the only curative therapy, especially for patients with intractable hemolysis or bone marrow failure. The aim of this study is to evaluate the outcomes of allogeneic HCT in PNH patients with aplastic anemia (PNH-AA) or without. Aplastik Aneminin Eşlik Ettiği ve Etmediği Paroksismal Noktürnal Hemoglobinüri Hastalarında Hematopoetik Kök Hücre Nakli Deneyimi: Çok Merkezli Türkiye DeneyimiFergün Yılmaz1, Nur Soyer2, Güldane Cengiz Seval3, Sinem Civriz Bozdağ4, Pervin Topcuoğlu3, Ali Ünal5, Leylagül Kaynar6, Gökhan Özgür7, Gülsan Türköz Sucak8, Hakan Göker9, Mustafa Velet9, Hakan Özdoğu10, Mehmet Yılmaz11, Emin Kaya12, Ozan Salim13, Burak Deveci14, İhsan Karadoğan14, Güray Saydam2, Fahri Şahin2, Filiz Vural21Marmara University School of Medicine, Department of Hematology, Istanbul, Turkey;PESG, PNH and Education Study Group2Ege University School of Medicine, Department of Hematology, Izmir, Turkey;PESG, PNH and Education Study Group 3Ankara University School of Medicine, Department of Hematology, Ankara, Turkey 4Ankara University School of Medicine, Department of Hematology, Ankara, Turkey;PESG, PNH and Education Study Group 5Erciyes University School of Medicine, Department of Hematology, Ankara, Turkey;PESG, PNH and Education Study Group 6Erciyes University School of Medicine, Department of Hematology, Ankara, Turkey 7Medical Park, Bahcesehir Hospital, Hematology and Transplantation Clinic, Istanbul, Turkey; PESG, PNH and Education Study Group 8Medical Park, Bahcesehir Hospital, Hematology and Transplantation Clinic, Istanbul, Turkey 9Hacettepe University School of Medicine, Department of Hematology, Ankara, Turkey 10Baskent University School of Medicine, Department of Hematology, Adana Bone Marrow Transplantation Center, Adana, Turkey 11SANKO University School of Medicine, Department of Hematology, Gaziantep, Turkey;PESG, PNH and Education Study Group 12İnönü University School of Medicine, Department of Hematology, Malatya, Turkey;PESG, PNH and Education Study Group 13Akdeniz University, School of Medicine, Department of Hematology, Antalya, Turkey; PESG, PNH and Education Study Group 14Medstar Antalya Hospital Bone Marrow Transplantation Center / Istanbul Gelisim University, Department of Hematology, Antalya, Turkey Amaç: Paroksismal noktürnal hemoglobunüri (PNH) hastalarının tedavisinde kompleman sisteminin değişik basamaklardaki inhibisyonu umut vadedici bir tedavi yöntemidir. Ancak hastalığın belirti ve bulgularını kontrol etmekte etkin bir tedavi yöntemi olsa da küratif bir tedavi seçeneği değildir. Özellikle kompleman inhibisyonuna rağmen devam eden hemolizi, replasman ihtiyacı ve kemik iliği yetmezliği ile birlikteliği olan hasta grubunda halen tek küratif tedavi seçeneği allojeneik kök hücre naklidir (KHN). Bu çalışmamızda aplastik anemi ile birlikteliği olan (PNH-AA) ve olmayan PNH hastalarında yapılmış allojeneik KHN’nin sonuçlarını değerlendirmeyi amaçladık. Fergün Yılmaz, Nur Soyer, Güldane Cengiz Seval, Sinem Civriz Bozdağ, Pervin Topcuoğlu, Ali Ünal, Leylagül Kaynar, Gökhan Özgür, Gülsan Türköz Sucak, Hakan Göker, Mustafa Velet, Hakan Özdoğu, Mehmet Yılmaz, Emin Kaya, Ozan Salim, Burak Deveci, İhsan Karadoğan, Güray Saydam, Fahri Şahin, Filiz Vural. Hematopoietic Stem Cell Transplantation for Patients with Paroxysmal Nocturnal Hemoglobinuria with or without Aplastic Anemia: A Multicenter Turkish Experience. . 2021; 38(3): 195-203 Corresponding Author: Fergün Yılmaz |
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