Treating iron overload in patients with non‐transfusion‐dependent thalassemia

Abstract

Despite receiving no or only occasional blood transfusions, patients with non-transfusion-dependent thalassemia (NTDT) have increased intestinal iron absorption and can accumulate iron to levels comparable with transfusion-dependent patients. This iron accumulation occurs more slowly in NTDT patients compared to transfusion-dependent thalassemia patients, and complications do not arise until later in life. It remains crucial for these patients' health to monitor and appropriately treat their iron burden. Based on recent data, including a randomized clinical trial on iron chelation in NTDT, a simple iron chelation treatment algorithm is presented to assist physicians with monitoring iron burden and initiating chelation therapy in this group of patients. Am. J. Hematol. 88:409–415, 2013. © 2013 Wiley Periodicals, Inc.

Serum ferritin level and morbidity risk in transfusion-independent patients with -thalassemia intermedia: the ORIENT study

“…2,5,6,9 When liver iron concentration measurement is unavailable, serum ferritin levels of 800 and 300 ng/mL can be used as an alternative to the 5 and 3 mg Fe/g dw liver iron concentration values, respectively, 2,5,6 as established in the THALASSA trial through correlation analysis between both iron overload indices.…”

“…Studies that evaluate the risk/benefit of initiating treatment in patients with a less conservative threshold (<800 ng/mL) would also be welcomed. 3 Mehran Karimi, 4 Amal El-Beshlawy, 5 Giovanna Graziadei, 1 Matthew Magestro, 6 Jerome Wulff, 7 Guilhem Pietri, 7 and Ali T. Taher …”

“…2,5,6 This threshold was primarily selected in the light of its established association with morbidity in β-thalassemia intermedia patients, 7,8 as well as recent evidence on the efficacy and safety of iron chelation therapy in non-transfusion-dependent thalassemia (including β-thalassemia intermedia) patients for whom treatment was started at 5 mg Fe/g dw or over (THALASSA trial).…”

Hepcidin and β-thalassemia major

“…Although erythrocyte transfusions are frequently the predominant source of iron (each milliliter of packed erythrocytes contains about 1 mg of iron), it is now widely appreciated that even non-transfusion-dependent thalassemia patients often develop lethal iron overload. 2 The discovery of the pathological suppression of the iron-regulatory hormone hepcidin in b-thalassemia and other iron-loading anemias [3][4][5] provided an explanation for these counterintuitive observations. Hepcidin deficiency allows increased intestinal iron absorption, often to rates similar to those in severe hereditary hemochromatosis.…”

Anti-oxidant properties and anti-hemolytic activity of Psidium guajava, Pandanous odorus and Rhinacanthus nasutus

“…In patients with β-thalassemia, sickle cells anemia and renal disease, ROS production is higher than normal (Nematbakhsh et al, 2013;Shazia et al, 2012). Moreover, iron overload has been observed in these patients (Finberg, 2012;Nematbakhsh et al, 2013;Taher et al, 2013). Thus, it can increase the susceptibility to peroxidation and induce hemolysis.…”