Wing-Yan Leung, Tracy Yuen-Han Lee, Yu-Yan Hwang, Jasmine Tsz-Man Lee, Chun-Fung Sin, Sio-Peng Mok, Chung-Yin Ha, Allen Li, Wendy Wai-Man Lam, Man-Fung Yuen, Yok-Lam Kwong, Dorine W Swinkels, Coby M Laarakkers, Daniel Ka-Leung Cheuk, Godfrey Chi-Fung Chan, Chak-Ho Li, Yiu-Fai Cheung, Chi Chiu So, Shau-Yin Ha, Anthony Pak-Yin Liu
{"title":"中国非输血依赖型地中海贫血患者的铁负荷和发病率。","authors":"Wing-Yan Leung, Tracy Yuen-Han Lee, Yu-Yan Hwang, Jasmine Tsz-Man Lee, Chun-Fung Sin, Sio-Peng Mok, Chung-Yin Ha, Allen Li, Wendy Wai-Man Lam, Man-Fung Yuen, Yok-Lam Kwong, Dorine W Swinkels, Coby M Laarakkers, Daniel Ka-Leung Cheuk, Godfrey Chi-Fung Chan, Chak-Ho Li, Yiu-Fai Cheung, Chi Chiu So, Shau-Yin Ha, Anthony Pak-Yin Liu","doi":"10.1111/bjh.20212","DOIUrl":null,"url":null,"abstract":"<p><p>Non-transfusion-dependent thalassaemia (NTDT) is associated with chronic health problems. A cross-sectional study was conducted in Hong Kong Chinese patients to evaluate the presentation and complications of NTDT. One hundred and nine patients aged between 6.7 and 72.3 years (median: 32 years), including 97 with α-thalassaemia intermedia and 12 with β-thalassaemia intermedia, were included from two public hospitals. Among them, 82% had never been transfused and 18% had received sporadic transfusions. Chelation therapy had been given to 7% of patients. Notably, 52% of patients had chronic health deficits with a strong positive correlation with advanced age, log-transformed ferritin levels and history of splenectomy. Hepatic iron overload was detected in 43% of patients, and extramedullary haematopoiesis in 14%. At least one endocrinopathy developed in 12% of patients. Patients with non-deletional α-thalassaemia intermedia and β-thalassaemia intermedia exhibited more severe disease than those with deletional α-thalassaemia intermedia. This study highlighted the need for ongoing monitoring and management strategies for NTDT to mitigate chronic health issues and improve outcomes.</p>","PeriodicalId":135,"journal":{"name":"British Journal of Haematology","volume":" ","pages":""},"PeriodicalIF":3.8000,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Iron overload and morbidities in Chinese with non-transfusion-dependent thalassaemia.\",\"authors\":\"Wing-Yan Leung, Tracy Yuen-Han Lee, Yu-Yan Hwang, Jasmine Tsz-Man Lee, Chun-Fung Sin, Sio-Peng Mok, Chung-Yin Ha, Allen Li, Wendy Wai-Man Lam, Man-Fung Yuen, Yok-Lam Kwong, Dorine W Swinkels, Coby M Laarakkers, Daniel Ka-Leung Cheuk, Godfrey Chi-Fung Chan, Chak-Ho Li, Yiu-Fai Cheung, Chi Chiu So, Shau-Yin Ha, Anthony Pak-Yin Liu\",\"doi\":\"10.1111/bjh.20212\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Non-transfusion-dependent thalassaemia (NTDT) is associated with chronic health problems. A cross-sectional study was conducted in Hong Kong Chinese patients to evaluate the presentation and complications of NTDT. One hundred and nine patients aged between 6.7 and 72.3 years (median: 32 years), including 97 with α-thalassaemia intermedia and 12 with β-thalassaemia intermedia, were included from two public hospitals. Among them, 82% had never been transfused and 18% had received sporadic transfusions. Chelation therapy had been given to 7% of patients. Notably, 52% of patients had chronic health deficits with a strong positive correlation with advanced age, log-transformed ferritin levels and history of splenectomy. Hepatic iron overload was detected in 43% of patients, and extramedullary haematopoiesis in 14%. At least one endocrinopathy developed in 12% of patients. Patients with non-deletional α-thalassaemia intermedia and β-thalassaemia intermedia exhibited more severe disease than those with deletional α-thalassaemia intermedia. This study highlighted the need for ongoing monitoring and management strategies for NTDT to mitigate chronic health issues and improve outcomes.</p>\",\"PeriodicalId\":135,\"journal\":{\"name\":\"British Journal of Haematology\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":3.8000,\"publicationDate\":\"2025-06-25\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"British Journal of Haematology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1111/bjh.20212\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"British Journal of Haematology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1111/bjh.20212","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"HEMATOLOGY","Score":null,"Total":0}
Iron overload and morbidities in Chinese with non-transfusion-dependent thalassaemia.
Non-transfusion-dependent thalassaemia (NTDT) is associated with chronic health problems. A cross-sectional study was conducted in Hong Kong Chinese patients to evaluate the presentation and complications of NTDT. One hundred and nine patients aged between 6.7 and 72.3 years (median: 32 years), including 97 with α-thalassaemia intermedia and 12 with β-thalassaemia intermedia, were included from two public hospitals. Among them, 82% had never been transfused and 18% had received sporadic transfusions. Chelation therapy had been given to 7% of patients. Notably, 52% of patients had chronic health deficits with a strong positive correlation with advanced age, log-transformed ferritin levels and history of splenectomy. Hepatic iron overload was detected in 43% of patients, and extramedullary haematopoiesis in 14%. At least one endocrinopathy developed in 12% of patients. Patients with non-deletional α-thalassaemia intermedia and β-thalassaemia intermedia exhibited more severe disease than those with deletional α-thalassaemia intermedia. This study highlighted the need for ongoing monitoring and management strategies for NTDT to mitigate chronic health issues and improve outcomes.
期刊介绍:
The British Journal of Haematology publishes original research papers in clinical, laboratory and experimental haematology. The Journal also features annotations, reviews, short reports, images in haematology and Letters to the Editor.