A substantial proportion of patients with transfusion-dependent beta-thalassemia major suffer from chronic liver disease. Iron overload resulting from repeated transfusions and HCV infection has been implicated in the development of liver fibrosis. Hepatic siderosis and fibrosis were assessed in 99 transfusion-dependent thalassemia (TDT) patients using transient elastography (TE) and liver iron concentration (LIC) assessed by T2*MRI at baseline and after 4 years. Data were analyzed retrospectively. At baseline, the overall mean liver stiffness measurement (LSM) was 7.4 ± 3.2 kPa and the mean LIC was 4.81 ± 3.82 mg/g dw (n = 99). Data available at 4 ± 1.5 years showed a significant reduction in LSM (6.6 ± 3.2 kPa, p 0.017) and hepatic siderosis measured by LIC (3.65 ± 3.45 mg/g dw, p 0.001). This result was confirmed when considering patients with iron overload at the time of the first measurement (n = 41) and subjects treated with a stable dose of deferasirox over the entire period of observation (n = 39). A reduction of LSM, yet not statistically significant, was achieved in patients on combined deferoxamine + deferiprone, while the group on deferoxamine (n = 11) remained stable over time. HCV-RNA positivity was found in 33 patients at T0, 20 of which were treated during the observation period. Patients who underwent anti-HCV therapy showed a more evident reduction in LSM (9 ± 3 vs 7 ± 3.1 kPa, p 0.016). Adequate chelation therapy is mandatory in order to prevent liver disease progression in TDT. Patients could benefit from regular non-invasive assessment of liver fibrosis by TE to indirectly monitor treatment adequacy and therapeutic compliance.
Progression of liver fibrosis can be controlled by adequate chelation in transfusion-dependent thalassemia (TDT) / D. Maira, E. Cassinerio, A. Marcon, M. Mancarella, M. Fraquelli, P. Pedrotti, M.D. Cappellini. - In: ANNALS OF HEMATOLOGY. - ISSN 0939-5555. - 96:11(2017 Nov), pp. 1931-1936.
|Titolo:||Progression of liver fibrosis can be controlled by adequate chelation in transfusion-dependent thalassemia (TDT)|
MAIRA, DILETTA (Primo)
|Parole Chiave:||chelation therapy; hepatic fibrosis; iron overload; magnetic resonance T2*; thalassemia major; transient elastography|
|Settore Scientifico Disciplinare:||Settore MED/09 - Medicina Interna|
|Data di pubblicazione:||nov-2017|
|Data ahead of print / Data di stampa:||5-set-2017|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1007/s00277-017-3120-9|
|Appare nelle tipologie:||01 - Articolo su periodico|