We assessed the value of pancreatic T2* magnetic resonance imaging (MRI) for predicting cardiac events from a large prospective database of transfusion-dependent thalassemia (TDT) patients. We considered 813 TDT patients (36.47 ± 10.71 years, 54.6% females) enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. MRI was used to measure hepatic, pancreatic, and cardiac iron overload (IO), to assess biventricular function and atrial dimensions, and to detect replacement myocardial fibrosis. The mean follow-up was 50.51 ± 19.75 months. Cardiac complications were recorded in 21 (2.6%) patients: one with heart failure (HF) and 20 with arrhythmias. The single patient who developed HF had, at the baseline MRI, a reduced pancreas T2*. Out of the 20 recorded arrhythmias, 17 were supraventricular. Pancreatic T2* values were a significant predictor of future arrhythmia-related events (hazard ratio = 0.89; p = 0.015). Pancreas T2* remained significantly associated with future arrhythmias after adjusting for any other univariate predictor (age and male sex, diabetes, history of previous arrhythmias, or left atrial area index). According to the receiver-operating characteristic curve analysis for arrhythmias, a pancreas T2* < 6.73 ms was the optimal cut-off value. In TDT, pancreatic iron levels had significant prognostic power for arrhythmias. Regular monitoring and the development of targeted interventions to manage pancreatic IO may help improve patient outcomes.

Background: Immune system impairment is frequently reported in patients affected by hemoglobinopathies due to various mechanisms, including iron accumulation, antigenic stimulation due to numerous transfusions, chronic hemolysis, and a general hyperinflammatory state. For these reasons, the antigenic immune response after a vaccine risks being ineffective. Methods: We evaluated the anti-spike IgG production after two doses of vaccine for SARS-CoV-2 in patients affected by hemoglobinopathies. Results: All 114 enrolled patients (100%) developed adequate antibody production, with a median value of serum IgG of 2184.4 BAU/mL (IQR 1127.4–3502.9). The amount of antibody was unrelated to any other clinical characteristics evaluated, including transfusion dependence or non-transfusion dependence, age, gender, disease type, ferritin, blood count, spleen status, and therapy with hydroxyurea or iron chelators (in all the cases p > 0.05). Moreover, 47 (41.2%) patients developed breakthrough SARS-CoV-2 infection during the first 2 years of follow-up after vaccination, all with a mildly symptomatic course, without requiring hospitalization or experiencing a significative drop in hemoglobin values, allowing for a slight delay in their transfusion regimen. Conclusion: Vaccination against COVID-19 is safe and effective for patients affected by hemoglobinopathies, ensuring adequate protection from severe infection.

Background. The E-MIOT (Extension-Myocardial Iron Overload in Thalassemia) project is an Italian Network assuring high-quality quantification of tissue iron overload by magnetic resonance imaging (MRI). We evaluated the impact of the COVID-19 pandemic on E-MIOT services. Methods. The activity of the E-MIOT Network MRI centers in the year 2020 was compared with that of 2019. A survey evaluated whether the availability of MRI slots for patients with hemoglobinopathies was reduced and why. Results. The total number of MRI scans was 656 in 2019 and 350 in 2020, with an overall decline of 46.4% (first MRI: 71.7%, follow-up MRI: 36.9%), a marked decline (86.9%) in the period March–June 2020, and a reduction in the gap between the two years in the period July–September. A new drop (41.4%) was recorded in the period October–December for two centers, due to the general reduction in the total amount of MRIs/day for sanitization procedures. In some centers, patients refused MRI scans for fear of getting COVID. Drops in the MRI services >80% were found for patients coming from a region without an active MRI site. Conclusions. The COVID-19 pandemic had a strong negative impact on MRI multi-organ iron quantification, with a worsening in the management of patients with hemoglobinopathies.

Objectives: Sickle Cell Disease (SCD) has a significant impact on the musculoskeletal system. The use of the Oxford Hip (OHS) and Shoulder score (OSS) as patient-reported outcome measures (PROMs) revealed a high rate of long-term impairment in joints with a pre-existing diagnosis of avascular necrosis (AVN). With this study, we aimed at detecting dysfunction in joints apparently not affected by AVN. Methods: This is a subgroup analysis of a previous core study assessing the OHS and OSS in 47 SCD patients with a pre-existing diagnosis of AVN. For this study, only patients with a pre-existing diagnosis of isolated AVN (only hip or only shoulder) were selected, and the OHS or OSS was measured in previously unaffected joints. Results: Among 37 patients with isolated AVN, 19 (51%) patients presented abnormal scores in the apparently unaffected joint; 16 (50%) patients with pre-existing isolated hip AVN had an abnormal OSS; and 9 (56%) had moderate to severe shoulder impairment. All patients with pre-existing isolated shoulder AVN had an abnormal OHS, with severe hip impairment in two out of three. As per clinical practice, patients with an abnormal score were prescribed joint magnetic resonance imaging (MRI) and/or X-rays. Only 10 out 19 (53%) performed imaging studies and all showed signs of AVN. Conclusions: Abnormal OHS and OSS values indicated a high rate of joint dysfunction in sites apparently not affected by AVN. The routine use of these PROMs should be applied to all SCD adults and subjects with a pathological score should have priority access to diagnostic radiological tests.