自身免疫性溶血性贫血:三级血液学中心的横断面研究

Wahinuddin Sulaiman, Jasram Singh Sidhu BALWANT SINGH, Nur Adlin Syafika SHAMZUL HISHAM, Nurul Syazwani Zakiah Marhassan, Siti Aishah Anuar, Kamini Kirubamoorthy
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摘要

自身免疫性溶血性贫血(AIHA)是一组由自身抗体引起失代偿性获得性溶血的疾病。马来西亚没有对自身免疫性溶血性贫血(AIHA)进行流行病学研究。本研究回顾性分析了马来西亚三级血液学中心包括埃文综合征在内的AIHA的流行病学。选择了1994年1月1日至2020年10月1日期间在怡保省拉贾·帕玛苏里·拜农医院血液科门诊诊断为AIHA和埃文综合征的18岁及以上患者。患者信息从门诊记录中检索。共纳入71例患者,其中以女性为主。男女的平均年龄具有可比性。种族分层显示,马来人的AIHA较高,其次是华人和印度人。温性AIHA患病率最高,为40.8%,冷性AIHA和埃文综合征患病率均为23.9%,混合性AIHA患病率为11.3%。原发性AIHA比继发性AIHA更常见,其次是埃文综合征。大约一半的继发性AIHA和继发性Evan’s综合征是由SLE引起的。总体而言,67.6%的患者仅接受皮质类固醇治疗,28.2%的患者联合使用免疫抑制剂。年龄越大的个体和女性患AIHA和Evan 's综合征的风险越大。发病率最高的是马来族。原发性温性AIHA是最常见的类型,大多数埃文综合征继发于自身免疫性疾病。
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Autoimmune Haemolytic Anaemia: A cross sectional study in a Tertiary Haematological Centre
Autoimmune haemolytic anaemia (AIHA) is a group of disorders wherein autoantibody causes decompensated acquired haemolysis. There has been no epidemiological study of autoimmune haemolytic anaemia (AIHA) in Malaysia. This study retrospectively analysed the epidemiology of AIHA including Evan’s Syndrome in a Tertiary Haematology Centre in Malaysia. Patients diagnosed with AIHA and Evan’s Syndrome at 18 years old and above between 1 January 1994 to 1 October 2020 at the out-patient Haematology Clinic of Hospital Raja Permaisuri Bainun, Ipoh were selected. Patients’ information was retrieved from the outpatient clinic records. A total of 71 patients were included of which predominantly female. The mean age for both genders were comparable. Ethnic stratification revealed AIHA was higher in Malays followed by Chinese and Indian. Warm AIHA was most prevalent at 40.8%, compared to cold AIHA and Evan’s Syndrome (both 23.9%), and mixed AIHA (11.3%). Primary was more common than secondary AIHA followed by Evan’s Syndrome. Approximately half of the secondary AIHA and secondary Evan’s Syndrome were due to SLE. Overall, 67.6% of patients received corticosteroid only and 28.2% combined with immunosuppressant. Individuals at higher age and females have higher risk of developing AIHA and Evan’s Syndrome. The highest prevalence was seen among the Malay ethnic. Primary warm AIHA is the most common type and majority of Evan’s syndrome are secondary to autoimmune diseases.
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