Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Srinagarind Hospital, Khon Kaen University, Khon Kaen, Thailand.
PLoS One. 2019 Mar 20;14(3):e0214148. doi: 10.1371/journal.pone.0214148. eCollection 2019.
Disease-related complications and management are different among patients with thalassemia. This study was aimed to review the prevalence, clinical risk factors for the complications and the management in patients with thalassemia in Thailand. A multicenter cross-sectional study was conducted in patients with thalassemia aged ≥ 18 years old. Thalassemia-related complications and management were reviewed. The clinical parameters significantly associated with the complications were analyzed by logistic regression methods. The prevalence of thalassemia-related complications was 100% in patients with transfusion-dependent thalassemia (TDT) and 58.8% in patients with non-transfusion-dependent thalassemia (NTDT). Advanced age was statistically associated with extramedullary hematopoiesis in both TDT and NTDT patients. Splenectomy was a significant risk factor for pulmonary hypertension in both groups of patients. Severe iron overload started earlier in patients with TDT than NTDT and was associated with diabetes mellitus (adjusted odds ratio (AOR) = 6.2, p-value = 0.02). Disease-related complications are more prevalent in patients with TDT than patients with NTDT. Splenectomy and advanced age were important risk factors for developing major complications in both groups. Early screening and management for specific disease-related complications should be considered in patients with thalassemia according to their clinical risk factors.
在患有地中海贫血症的患者中,疾病相关的并发症和管理方式存在差异。本研究旨在回顾泰国地中海贫血症患者的患病率、并发症的临床危险因素和管理方法。一项多中心横断面研究纳入了年龄≥18 岁的地中海贫血症患者。回顾了地中海贫血症相关并发症和管理方法。通过逻辑回归方法分析了与并发症显著相关的临床参数。输血依赖性地中海贫血症(TDT)患者的地中海贫血症相关并发症患病率为 100%,非输血依赖性地中海贫血症(NTDT)患者的患病率为 58.8%。在 TDT 和 NTDT 患者中,年龄较大与骨髓外造血相关。脾切除术是两组患者发生肺动脉高压的显著危险因素。在 TDT 患者中,严重铁过载的起始时间早于 NTDT 患者,且与糖尿病相关(校正优势比(AOR)=6.2,p 值=0.02)。TDT 患者的疾病相关并发症比 NTDT 患者更为常见。脾切除术和年龄较大是两组患者发生主要并发症的重要危险因素。根据患者的临床危险因素,应考虑对地中海贫血症患者进行特定疾病相关并发症的早期筛查和管理。