Retrospective analysis of hemophilia B in Turkey: identifying main characteristics and treatment options.

Autor: Zülfikar B; Hereditary Bleeding Disorders Unit, Istanbul University Oncology Institute, Istanbul, Turkey., Koç B; Hereditary Bleeding Disorders Unit, Istanbul University Oncology Institute, Istanbul, Turkey., Şahin F; Department of Internal Diseases, Division of Hematology, Ege University Medical Faculty Hospital, Izmir, Turkey., Şaşmaz Hİ; Department of Pediatric Hematology, Cukurova University Faculty of Medicine, Adana, Turkey., Kavaklı K; Department of Children's Health and Diseases, Division of Pediatric Hematology, Ege University Medical Faculty Hospital, Izmir, Turkey., Balkan C; Department of Children's Health and Diseases, Division of Pediatric Hematology, Ege University Medical Faculty Hospital, Izmir, Turkey., Antmen AB; Department of Pediatric Hematology and Oncology, Acibadem Hospital, Adana, Turkey., Akbayram S; Department of Pediatric Hematology and Oncology, Gaziantep University Medical Faculty, Gaziantep, Turkey., Güvenç B; Department of Internal Diseases, Division of Hematology, Cukurova University Faculty of Medicine, Adana, Turkey., Okan V; Department of Internal Diseases, Division of Hematology, Gaziantep University Medical Faculty, Gaziantep, Turkey., Türkkan E; Department of Pediatric Hematology-Oncology, Ministry of Health Prof. Dr. Cemil Taşçıoğlu City Hospital, Istanbul, Turkey., Albayrak C; Department of Pediatric Hematology and Oncology, Ondokuz Mayıs University Faculty of Medicine, Samsun, Turkey., Albayrak D; Department of Pediatric Hematology-Oncology, Samsun Medical Park Hospital, Samsun, Turkey., Sarper N; Department of Pediatric Hematology, Kocaeli University School of Medicine, Kocaeli, Turkey., Celkan TT; Department of Pediatric Hematology and Oncology, İstinye University, Istanbul, Turkey., Ayyıldız O; Department of Internal Medicine, Division of Hematology, Dicle University Medical Faculty, Diyarbakır, Turkey., Aksu S; Department of Internal Medicine, Division of Hematology, Hacettepe University Faculty of Medicine, Ankara, Turkey., Patıroğlu T; Division of Pediatric Immunology and Oncology, Losante Children's and Adult Hospital, Ankara, Turkey., Şalcıoğlu Z; Department of Pediatric Hematology, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey., Güneş AM; Department of Pediatric Hematology and Oncology, Uludağ University Faculty of Medicine, Bursa, Turkey., Torun YA; Department of Pediatric Hematology and Oncology, İstinye University, Istanbul, Turkey., Çalışkan Ü; Department of Pediatric Hematology, Necmettin Erbakan University Meram Medical Faculty Hospital, Konya, Turkey., Tokgöz H; Department of Pediatric Hematology, Necmettin Erbakan University Meram Medical Faculty Hospital, Konya, Turkey., Ay Y; Department of Pediatric Hematology, Pamukkale University School of Medicine, Denizli, Turkey., Özdemir GN; Department of Pediatric Hematology and Oncology, İstinye University, Istanbul, Turkey., Sönmez M; Department of Internal Medicine, Division of Hematology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey., Ünal E; Department of Pediatrics, Division of Pediatric Hematology and Oncology, Erciyes University Faculty of Medicine, Kayseri, Turkey., Öner AF; Department of Pediatric Hematology and Oncology, Yuzuncu Yil University Faculty of Medicine, Van, Turkey., Güler N; Department of Internal Medicine, Division of Hematology, Pamukkale University School of Medicine, Denizli, Turkey., Küpesiz OA; Department of Pediatric Hematology, Akdeniz University Faculty of Medicine, Antalya, Turkey., Ören H; Department of Pediatrics, Division of Pediatric Hematology, Dokuz Eylul University Faculty of Medicine, İzmir, Turkey., Karaman S; Department of Pediatrics, Division of Pediatric Hematology-Oncology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey., Ünüvar A; Department of Pediatrics, Division of Pediatric Hematology-Oncology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey., Dağlı M; Department of Internal Medicine, Division of Hematology, Selcuk University Faculty of Medicine, Konya, Turkey., Demir AM; Department of Internal Medicine, Division of Hematology, Trakya University Faculty of Medicine, Edirne, Turkey., Söker M; Department of Pediatric Hematology, Dicle University Medical Faculty, Diyarbakır, Turkey., Alioğlu B; Department of Pediatric Hematology and Oncology, Ankara Training and Research Hospital, Ankara, Turkey., Kaya Z; Department of Pediatric Hematology, Gazi University Faculty of Medicine, Ankara, Turkey., Ayhan AC; Department of Pediatric Oncology and Hematology, Istanbul Medeniyet University Faculty of Medicine, Istanbul, Turkey., Bıçakçı Z; Department of Pediatric Hematology and Oncology, Atatürk University, Erzurum, Turkey., Aral YZ; Department of Pediatric Hematology, Adnan Menderes University School of Medicine, Aydın, Turkey., Ar MC; Department of Internal Medicine, Division of Hematology, Istanbul University - Cerrahpasa, Cerrahpasa Faculty of Medicine, Istanbul, Turkey.
Jazyk: angličtina
Zdroj: Research and practice in thrombosis and haemostasis [Res Pract Thromb Haemost] 2024 Oct 10; Vol. 8 (7), pp. 102588. Date of Electronic Publication: 2024 Oct 10 (Print Publication: 2024).
DOI: 10.1016/j.rpth.2024.102588
Abstrakt: Background: Hemophilia B (HB), an X-linked recessive inherited bleeding disorder, exhibits a high prevalence among males.
Objectives: To present the first national cohort of persons with HB to define the demographics, clinical characteristics, and treatment patterns in Turkey.
Methods: This multicenter, retrospective study included 433 alive persons with HB registered in 35 centers between 1961 and 2018. Analyses were performed by age subgroups (0-17 years, 18-64 years, and ≥65 years), disease severity by factor levels (severe, <1 IU/dL; moderate, 1-5 IU/dL; mild, >5 IU/dL). Additionally, patients were stratified based on the initiation year of follow-up at the relevant study center, creating 2 periods: 1993-2006 (referred to as period A) and 2007-2018 (referred to as period B).
Results: Predominantly male (98.6%), the median age at data entry was 22.1 years ( n  = 429). The majority (49.0%) had moderate HB, followed by severe (30.0%) and mild (15.7%) disease. Of the 377 patients with complete treatment details, 209 (55.4%) were under prophylaxis from their diagnosis onwards, while 79 patients (21.0%) only received on-demand treatment. Additionally, 89 patients (23.6%) initially underwent on-demand treatment and later were switched to prophylaxis. Knees were the primary site of bleeding and the most frequently intervened joints. Most of the major (47.5%) and minor (53.3%) orthopedic procedures were carried out in persons with severe HB, while half of radioactive synovectomy procedures were performed on persons with moderate HB.
Conclusion: This paper describes the demographics, clinical characteristics, and treatments patterns of a large cohort of alive persons with HB on a national scale.
(© 2024 The Authors.)
Databáze: MEDLINE
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