A multicentre, retrospective study of epidemiology and outcome of aplastic anaemia among adult population in Sabah and Sarawak from year 2006 to 2017.

Q3 Medicine Medical Journal of Malaysia Pub Date : 2024-11-01

G W C Lee, M Y Yeap, X Y Tan, A S O Tang, Y F Ho, K B Law, S W F Lee, L P Chew, L L L Wong

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引用次数: 0

Abstract

Introduction: Aplastic anaemia (AA) is a rare disorder of bone marrow failure, characterized by bone marrow hypocellularity with pancytopenia. The annual incidence rates of AA in Asia are observed to be two to three times higher than Europe and North America. Since the introduction of immunosuppressive therapy (IST) and of allogenic stem cell transplant (SCT), the outcome of severe AA has significantly improved. We conducted a 12-year multi-centre retrospective study among the adult AA population in Sabah and Sarawak.

Materials and methods: A total of 119 AA patients had been identified from hospital records of the involved sites, namely Queen Elizabeth Hospital in Sabah, Sarawak General Hospital, Sibu Hospital, Miri Hospital and Bintulu Hospital in Sarawak from Jan 2006 to Dec 2017.

Results: The median age at diagnosis was 46 years, and native ethnic group from Sabah, Kadazan-Dusun, recorded the highest percentage of 41.2%, which could be explained by higher frequency of HLA-DRB1*15:01, an alelle linked to increased risk of AA, among this ethnic group. The majority of patients (59.7%) received cyclosporine (CsA) as monotherapy or in combination with other non-IST agents such as danazol, which was instituted in 48.7% of the patients, while a third of them (33.7%) received antithymocyte globulin (ATG) therapy with or without CsA, and 12.4% underwent allogenic SCT. The five-year overall survival (OS) for all AA patients was 76.1%. Elderly patients >60 years old and those with severe disease had more inferior 5-year survival.

Conclusion: A prospective study is warranted to determine the true incidence rate, epidemiological distributions, treatment outcome and overall survival of AA patients in Malaysia. Establishment of allogenic SCT in East Malaysia is imperative to make this curative therapy more accessible to patients with severe disease and improve the outcome.

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2006年至2017年沙巴和沙捞越成年人口再生障碍性贫血流行病学和结果的多中心回顾性研究。

再生障碍性贫血（AA）是一种罕见的骨髓衰竭疾病，以骨髓细胞减少伴全血细胞减少为特征。据观察，亚洲的AA年发病率比欧洲和北美高2至3倍。自从引入免疫抑制疗法（IST）和同种异体干细胞移植（SCT）以来，严重AA的预后有了显著改善。我们对沙巴和沙捞越的成年AA人群进行了为期12年的多中心回顾性研究。材料与方法：从2006年1月至2017年12月沙巴伊丽莎白女王医院、砂拉越总医院、西武医院、美里医院和砂拉越民都鲁医院的病历中共鉴定出119例AA患者。结果：确诊时中位年龄为46岁，卡达山-杜松沙巴州本地族群比例最高，为41.2%，这可能与该族群中与AA风险增加相关的HLA-DRB1*15:01等位基因频率较高有关。大多数患者（59.7%）接受环孢素（CsA）单药治疗或与其他非ist药物（如达那唑）联合治疗，48.7%的患者采用环孢素，而三分之一的患者（33.7%）接受抗胸腺细胞球蛋白（ATG）治疗，有或没有CsA， 12.4%接受同种异体SCT。所有AA患者的5年总生存率（OS）为76.1%。60岁以下的老年患者和病情严重的患者5年生存率较低。结论：有必要进行前瞻性研究，以确定马来西亚AA患者的真实发病率、流行病学分布、治疗结果和总生存率。在东马来西亚建立同种异体SCT是必要的，以使这种治疗方法更容易获得严重疾病的患者和改善结果。

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来源期刊

Medical Journal of Malaysia Medicine-Medicine (all)

CiteScore

1.20

自引率

0.00%

发文量

165

期刊介绍： Published since 1890 this journal originated as the Journal of the Straits Medical Association. With the formation of the Malaysian Medical Association (MMA), the Journal became the official organ, supervised by an editorial board. Some of the early Hon. Editors were Mr. H.M. McGladdery (1960 - 1964), Dr. A.A. Sandosham (1965 - 1977), Prof. Paul C.Y. Chen (1977 - 1987). It is a scientific journal, published quarterly and can be found in medical libraries in many parts of the world. The Journal also enjoys the status of being listed in the Index Medicus, the internationally accepted reference index of medical journals. The editorial columns often reflect the Association''s views and attitudes towards medical problems in the country. The MJM aims to be a peer reviewed scientific journal of the highest quality. We want to ensure that whatever data is published is true and any opinion expressed important to medical science. We believe being Malaysian is our unique niche; our priority will be for scientific knowledge about diseases found in Malaysia and for the practice of medicine in Malaysia. The MJM will archive knowledge about the changing pattern of human diseases and our endeavours to overcome them. It will also document how medicine develops as a profession in the nation. We will communicate and co-operate with other scientific journals in Malaysia. We seek articles that are of educational value to doctors. We will consider all unsolicited articles submitted to the journal and will commission distinguished Malaysians to write relevant review articles. We want to help doctors make better decisions and be good at judging the value of scientific data. We want to help doctors write better, to be articulate and precise.