Reduction of Liver Iron Load in Adult Patients with β-Thalassemia Major Treated with Modern Chelation Modalities

Abstract

Background: Management of beta-thalassemia major (TM) requires life-long hemotransfusions leading to iron overload. Iron elimination is enhanced by the use of modern chelators.  Aim: To assess the effect of modern chelation therapy by dynamics of serum ferritin concentration and liver MRI T2*. Patients and methods: Forty-six patients with TM (male to female ratio =1:1, mean age 33.2±10.9 years) were prospectively studied between 2011 and 2014. Twenty-one patients (45.7%) were treated with deferasirox, 17 (37%) – with deferiprone, and 8 (17.3%) – with deferiprone in combination with deferoxamine. Ferritin was measured by ELISA. MRI T2* was assessed by Siemens Magnetom Avanto 1.5T. The patients were allocated into 3 groups based on their initial ferritin level and liver MRI T2*. Statistical analysis was performed using SPSS v. 18 for Windows. Data were analysed by descriptive analysis, analysis of variance and correlative analysis, means were compared using t-test and one-way ANOVA. Results: In 2011, 9 (19.5%) patients had normal liver MRI T2*; in 2014 they were 17 (37%). The patients with mild grade liver siderosis were 12 (26%) in 2011, and in 2014 they were 14 (30.4%). In 2011, the patients with moderate liver siderosis were 14 (30.4%), and in 2014 – 12 (26.0%). Eleven patients (23.9%) had severe liver siderosis in 2011 and only two patients (4.0%) were diagnosed with the condition in 2014. Conclusion: A reduction of iron overload was found in all studied groups. This positive effect is attributed to the use of modern chelators and the ease of access to accurate monitoring.

Чернодробни Усложнения При SS-Таласемия

“…Методът измерва желязото в мг на грам суха чернодробна тъкан и определя риска от органна увреда. Стойности над 1.6 mg/g чернодробна тъкан са високи; < 7 mg/g имат нисък риск, 7-15 mg/g умерен риск, ≥ 15 mg/g има висок риск от развитие на чернодробна фиброза и цироза [8].…”

“…В тези случаи MRI T2* е много полезен [9]. Проследяването с MRI T2* е неинвазивно и помага за оптимизиране на хелаторната терапия, по-малко усложнения и подобрена преживяемост [8,13]. През 2012 г. Клиничната мрежа за проучване на таласемия излиза с препоръки за извършване на MRI T2* веднъж годишно за мониториране на желязното натрупване в черния дроб и сърцето при деца над 10-годишна възраст [14].…”