Rafsanjani Khadijeh Arjmandi, Mafi Negar, Tafreshi R Isa
Departments of Pediatric Hematology-Oncology and Pediatric Cardiology, Ali Asghar Children's Hospital, Tehran University of Medical Sciences, Tehran, Iran. khadijeh
Pediatr Hematol Oncol. 2011 Sep;28(6):497-508. doi: 10.3109/08880018.2011.572144. Epub 2011 Jul 5.
Patients with thalassemia intermedia (TI) experience many complications, of which the incidence varies greatly among cases. Considering the high prevalence of thalassemia in Iran, the study was carried out to determine the frequency of TI complications in Iranian patients and to find possible risk factors for each of them. Using the sampling method of "census," the authors included 153 patients who were seen in their tertiary hematology clinic with the diagnosis of TI during 1996-2010; an analytical cross-sectional study was performed and the data was analyzed by SPSS software using univariate and regression analyses. Mean age of the patients at the time of the study was 17.4 years and 36.5% were receiving transfusions (regularly or occasionally). Mean hemoglobin was 9.2 g/dL and mean serum ferritin was 858 ng/mL. Splenectomy was performed in 46.9% and it was correlated with age and the age at diagnosis in regression analysis. Cholelithiasis was found in 25.5% and was correlated with age and history of splenectomy. Pulmonary hypertension, detected in 23.5%, was correlated with thrombocytosis and mitral valve regurgitation in univariate analysis. Endocrine disease (hypogonadism, hypothyroidism, and adrenal insufficiency) was detected in 8% of the patients. In univariate analysis, endocrine disease was correlated with age of the patients. Regarding bone density of the spine, 53% of cases had osteoporosis. Thrombocytosis was present in 42% of patients and was correlated with their age. Since the severity of thalassemia intermedia vary greatly among patients, a careful evaluation of clinical, laboratory, and genetic aspects is necessary to differentiate TI in a patient at presentation. Moreover, TI patients should be carefully followed up for early detection and management of newly developed complications. The authors also suggest confirmatory controlled studies with larger sample sizes to assist in developing guidelines for surveillance and treatment of TI.
中间型地中海贫血（TI）患者会出现多种并发症，其发生率在不同病例中差异很大。鉴于伊朗地中海贫血的高患病率，开展了这项研究，以确定伊朗患者中TI并发症的发生率，并找出每种并发症可能的危险因素。作者采用“普查”抽样方法，纳入了1996年至2010年间在其三级血液学诊所被诊断为TI的153例患者；进行了一项分析性横断面研究，并使用SPSS软件通过单变量和回归分析对数据进行分析。研究时患者的平均年龄为17.4岁，36.5%的患者正在接受输血（定期或偶尔）。平均血红蛋白为9.2 g/dL，平均血清铁蛋白为858 ng/mL。46.9%的患者进行了脾切除术，在回归分析中，这与年龄和诊断时的年龄相关。25.5%的患者发现有胆结石，这与年龄和脾切除病史相关。在单变量分析中，23.5%的患者检测出肺动脉高压，这与血小板增多症和二尖瓣反流相关。8%的患者检测出内分泌疾病（性腺功能减退、甲状腺功能减退和肾上腺功能不全）。在单变量分析中，内分泌疾病与患者年龄相关。关于脊柱骨密度，53%的病例患有骨质疏松症。42%的患者存在血小板增多症，这与他们的年龄相关。由于中间型地中海贫血患者的严重程度差异很大，在患者就诊时仔细评估临床、实验室和基因方面对于鉴别TI很有必要。此外，应对TI患者进行仔细随访，以便早期发现和处理新出现的并发症。作者还建议开展样本量更大的验证性对照研究，以协助制定TI的监测和治疗指南。
