Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions

Abstract

BACKGROUND-Transfusion therapy is frequently used to prevent morbidity in sickle cell disease (SCD), and subsequent iron overload is common. The objective of this study was to evaluate the current standard of care in monitoring iron overload and related complications in patients with SCD compared to thalassemia (Thal).

Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention

“…Transfusion duration was longer in those who underwent liver biopsy (8.2 vs. 5.7 years, P < 0.0001). This liver biopsy rate is strikingly similar to a rate of 46.5% in transfused pediatric patients with SCA in a prior report, which was significantly lower than the rate of 69.6% in transfused children with thalassemia [1]. The mean LIC at the time of past biopsy was 6.8 mg/g dw (n 5 73, range, 1.15-44.5) and had risen to 15.4 mg/g dw at study entry (mean, 2.6 years).…”

“…The mean transfusion duration prior to chelation initiation was 2.6 years (median, 2.0; range, 0-12.6), which is substantially lower than the duration of 4.1 years for sickle cell patients and 5.5 years for thalassemia patients in a prior report [1]. Treatment guidelines generally recommend initiating chelation after 10-20 transfusions have been administered [19], or after one to two years of transfusions, similar to the time of initiation of chelation in our overall population.…”

“…Management of transfusional iron overload in SCA has been reported as suboptimal [1], but studies characterizing monitoring and treatment practices for iron overload in children with SCA, particularly in recent years with the expansion of chelator options, are lacking. We investigated the degree of iron loading and treatment practices of 161 children with SCA receiving transfusions for a history of stroke who participated in the Stroke with Transfusions Changing to Hydroxyurea (SWiTCH) trial.…”

Assessing cardiac and liver iron overload in chronically transfused patients with sickle cell disease

“…Serum ferritin is a simple, widely available laboratory test that has been used to monitor iron overload in chronically transfused patients in different settings, including those with limited resources (Brittenham et al, 1993;Adamkiewicz et al, 2009;Kwiatkowski et al, 2012a;Porter & Garbowski, 2013;Ware & Kwiatkowski, 2013;El Beshlawy et al, 2014). Compared to patients with other transfusiondependent states, patients with SCD are significantly less likely to have had a liver biopsy for iron monitoring Fung et al, 2008) and, in a recent paediatric trial of chronically transfused children with SCD and a history of stroke, less than half had ever had a liver biopsy at study enrolment (Kwiatkowski et al, 2012a). Compared to patients with other transfusiondependent states, patients with SCD are significantly less likely to have had a liver biopsy for iron monitoring Fung et al, 2008) and, in a recent paediatric trial of chronically transfused children with SCD and a history of stroke, less than half had ever had a liver biopsy at study enrolment (Kwiatkowski et al, 2012a).…”

Transfusional Iron Overload in a Cohort of Children with Sickle Cell Disease: Impact of Magnetic Resonance Imaging, Transfusion Method, and Chelation

“…Patient and provider concerns about these risks contributed to low utilization rates at our center. Indeed, disparately low rates of assessment of LIC in patients with SCD compared with thalassemia have been described . The substitution of ferritin levels for assessment of iron burden in this patient population may lead to suboptimal iron management.…”