目的 探讨采用MRI T2*值定量评估重型β地中海贫血患者体内器官铁沉积的价值.方法 为回顾性病例分析.患者为经基因检测诊断为重型β地中海贫血,且完成了心脏和(或)腹部MRI检查,共102例患者,其中47例接受了脾切除手术.95例行心脏MRI扫描;102例行腹部肝脏、胰腺及肾脏扫描,55例行脾脏扫描.测量心脏、肝脏、脾脏、胰腺和肾脏T2*值,并测量肝铁浓度(LIC).81例行实验室血清铁蛋白(SF)测定.采用Spearman相关分析评价SF、LIC、年龄、各器官T2*值间的相关性.采用独立样本t检验(正态分布)或Mann-Whitney U检验(非正态分布)比较脾脏切除组与未切除组患者间肝脏、心脏、肾脏和胰腺T2*值的差异.结果 SF与LIC呈高度正相关,与年龄呈中度正相关,与肝脏及胰腺T2*值呈中度负相关,r值分别为0.833、0.537、-0.623和-0.403,P均〈0.05;SF与心脏、肾脏、脾脏T2*值无相关性(P〉0.05).肝脏T2*值与LIC呈高度负相关(r=-0.925,P〈0.01).各器官T2*值间,除了肝脏和脾脏、肝脏和胰腺间的T2*值呈中度相关(r值分别为0.634、0.530,P〈0.01),其他器官间的T2*值均无相关性(P〉0.05).LIC与年龄呈中度正相关(r=0.457,P〈0.01).脾脏切除组患者的肝脏、胰腺T2*值低于未切除组,肾脏T2*值高于未切除组,差异均有统计学意义(P均〈0.05);心脏T2*值差异无统计学意义(P〉0.05).结论 重型β地中海贫血患者容易发生心脏和腹部多器官的铁沉积过量,采用MRI技术定量测量T2*值可以反映体内铁过载情况.
Objective To explore the value of MRI T2*quantification of visceral iron deposition in patients with β-thalassemia major. Methods A total of one hundred and two patients with β-thalassemia major were retrospectively analyzed. They underwent genetic testing and the cardiac and (or) abdominal MRI scanning. Among them, 47 patients underwent splenectomy operation, 95 patients underwent cardiac MRI scanning, 102 underwent abdominal MRI scanning for their livers, pancreases and kidneys, and 55 underwent splenic MRI scanning. The T2* values of the hearts, livers, pancreases and kidneys were measured, meanwhile the liver iron concentrations (LIC) were measured. The levels of serum ferritin (SF) patients were measured in 81 patients. The Spearman rank correlation was employed to analyze the relationship between SF, LIC, age and each organs T2* values. Independent sample t test (normal distribution) or the Mann- Whitney U test (non-normal distribution) were used to compare the hepatic, cardiac, renal and splenic T2* values differences between patients with splenectomy and those without. Results Serum ferritin were highly positively correlated with LIC, moderately correlatied with age, moderately negatively correlated with hepatic and pancreatic T2* values, with r values of 0.833, 0.537,-0.623 and -0.403, respectively (P〈0.05). There was significantly negative correlation between hepatic T2*value and LIC (r=-0.925, P〈0.01). There were moderately correlations between hepatic and splenic T2*values (r=0.634, P〈0.01), hepatic and pancreatic T2*values (r=0.530, P〈0.01). However, those T2*values of other organs had no correlation (P〉0.05). The hepatic and pancreatic T2*values were reduced (P〈0.05), the renal T2* value was increased (P〈0.05) in the patients with splenectomy. The cardiac T2* value was not statistically different (P〉0.05). Conclusions The patients with β-thalassemia major have prone to suffer from excessive iron deposition