Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, 50200, Chiang Mai, Thailand.
Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, 50200, Chiang Mai, Thailand.
Ann Hematol. 2022 Aug;101(8):1667-1675. doi: 10.1007/s00277-022-04870-3. Epub 2022 May 23.
Reports of inflammatory rheumatic diseases (IRD) in thalassemia are limited. This study aimed to determine the prevalence and clinical characteristics of IRD in patients with thalassemia disease. Consecutive adult patients with thalassemia disease, confirmed by hemoglobin typing, attending the Hematology Clinic between June 2019 and May 2021 were invited to join this study. All of them had their history taken and a physical examination for IRD. Those with IRD had their medical records reviewed. Sixty-three patients (transfusion-dependent in 50) were included in this study. There was α-, β-, and co-inheritance of α- and β-thalassemia in 22.22%, 73.02%, and 4.76% of the patients, respectively, with β-thalassemia/Hb E disease in 53.97%. Twenty-three patients had IRD (rheumatoid arthritis in 9, gout in 6, systemic lupus erythematosus in 3, spondyloarthropathy in 2, and one patient each with dermatomyositis, overlap syndrome, and unclassified polyarthralgia). Clinical manifestations and laboratory findings were similar to IRD patients in general. In 40 patients without IRD, direct and indirect Coombs tests and antinuclear antibody (ANA) were positive in 51.72%, 27.59%, and 10.26%, respectively. When comparing among these 40 patients, between those with non-transfusion-dependent thalassemia (n = 10) and those with transfusion-dependent thalassemia (n = 30), the latter had non-significantly more positive direct Coombs (60.87% vs. 16.67%), indirect Coombs (30.43% vs. 16.67%), and ANA tests (13.33% vs. 0%). The prevalence of IRD in patients with thalassemia disease was rather high. Positive direct Coombs test and ANA were common in transfusion-dependent patients.
有关地中海贫血患者炎症性风湿性疾病（IRD）的报道有限。本研究旨在确定地中海贫血患者中 IRD 的患病率和临床特征。
2019 年 6 月至 2021 年 5 月期间，连续邀请在血液科诊所就诊的经血红蛋白分型证实的地中海贫血成年患者参加本研究。对所有患者进行病史采集和 IRD 体格检查。对患有 IRD 的患者进行病历回顾。
本研究共纳入 63 例患者（50 例依赖输血）。患者分别有 22.22%、73.02%和 4.76%为α-、β-和α-β 地中海贫血遗传，53.97%为β-地中海贫血/血红蛋白 E 疾病。23 例患者患有 IRD（9 例类风湿关节炎、6 例痛风、3 例系统性红斑狼疮、2 例脊柱关节病和 1 例皮肌炎、重叠综合征和未分类多关节炎）。临床表现和实验室检查与一般 IRD 患者相似。在 40 例无 IRD 的患者中，直接和间接 Coombs 试验和抗核抗体（ANA）阳性率分别为 51.72%、27.59%和 10.26%。在这 40 例患者中进行比较，在非输血依赖型地中海贫血（n=10）和输血依赖型地中海贫血（n=30）患者中，后者直接 Coombs 试验（60.87%比 16.67%）、间接 Coombs 试验（30.43%比 16.67%）和 ANA 试验（13.33%比 0%）阳性率非显著更高。地中海贫血患者中 IRD 的患病率相当高。输血依赖型患者中直接 Coombs 试验和 ANA 阳性较为常见。
