Predisposing factors for advanced liver fibrosis in patients with sickle cell disease.

Sickle cell disease (SCD) is one of the most common monogenic disorders worldwide and liver complications are common in this group of patients. Our study aims to highlight the prevalence of chronic liver complications and the main predisposing factors for advanced liver fibrosis in SCD patients. For this purpose, 219 patients from eight Thalassemia and Sickle Cell Units across Greece enrolled in our study and history of liver related disease complications was recorded, as well as a full laboratory and imaging analysis concerning their liver function. 13.6% of the patients had advanced liver fibrosis. The presence of liver fibrosis was significantly correlated with advanced age, male gender, cholelithiasis and higher LDH, γ-GT, INR, direct and indirect bilirubin levels. These patients had exhibited significantly more episodes of liver crises and acute intrahepatic cholestasis. No correlation was observed with right heart failure or previous viral hepatitis. Patients with advanced liver fibrosis were receiving a more intensive transfusion therapy for a longer period of time and had higher Liver Iron Concentration levels. Our study shows that liver complications and cirrhosis is a significant cause of morbidity in patients with SCD and it is primarily associated with intravascular hemolysis and vaso-occlusive phenomena and secondarily with iron overload.

Vaccination practices and knowledge among adults with hemoglobinopathies in Greece: a nationwide survey.

Background: Hemoglobinopathies, such as sickle cell disease and thalassemia, are genetic disorders that affect hemoglobin structure or production, leading to various health complications, including an increased risk of infections. Vaccinations play a crucial role in managing these conditions by providing essential protection against preventable diseases. Ensuring timely and appropriate immunizations is vital for reducing infection-related morbidity and improving the overall health and quality of life for affected individuals. Objectives: Our objective was to assess vaccination coverage, as well as knowledge, attitudes, and practices toward vaccination in Greek patients with hemoglobinopathies. Design and methods: A nationwide survey of hemoglobinopathy patients in Greece using a 37-item questionnaire was conducted anonymously via Google Forms. It covered demographics, previous vaccinations, vaccine-preventable infections, beliefs about vaccines, and antibiotic prophylaxis post-splenectomy. The survey was distributed through Thalassemia and Sickle Cell Units and organizations. Results: Participants were predominantly university-educated married women aged 30-50 years with transfusion-depended thalassemia (n = 149, 60.5%) or sickle cell anemia (n = 52, 21.1%). Reported childhood vaccination rates aligned with Greece's national immunization program. However, adult coverage was suboptimal across all age groups for measles (10%), varicella (27%), zoster (2% for over 50 years old individuals), hepatitis A (13.9% of those with chronic liver disease) and hepatitis B (41%), pneumococcal (81.3%), meningococcal (37%), tetanus (20.3%), and influenza (67.1%) vaccines compared to guidelines. Participants relied predominantly on healthcare providers for vaccine information but perceived limited engagement. Those over age 50 demonstrated lower adult vaccination rates and higher misconceptions compared to younger cohorts. Conclusion: Addressing educational and access gaps could help protect this vulnerable population. Our findings highlight the need for coordinated efforts to optimize adult immunization for those with hemoglobinopathies.

Vaccination Habits of Greek Adults with Hemoglobin Disorders We surveyed 246 adults in Greece with hemoglobin disorders like thalassemia and sickle cell anemia. Most were educated women aged 30­50. When it came to childhood vaccinations, they followed Greece's guidelines. But as adults, they weren't getting vaccinated enough. For instance, only a small percentage got vaccines for measles, varicella, hepatitis A and B, among others. They mostly relied on doctors for vaccine info but felt they weren't getting enough guidance. Older adults were less likely to get vaccinated and had more misconceptions. To help, we need better education and easier access to vaccines.

Real-world complication burden and disease management paradigms in transfusion-related ß-thalassaemia in Greece: Results from ULYSSES, an epidemiological, multicentre, retrospective cross-sectional study.

Patients with transfusion-dependent beta (ß)-thalassaemia experience a broad range of complications. ULYSSES, an epidemiological, multicentre, retrospective cross-sectional study, aimed to assess the prevalence and severity of treatment and disease complications, capture disease management and identify predictors of complications in patients with transfusion-dependent ß-thalassaemia, treated in routine settings in Greece. Eligible patients were adults diagnosed with ß-thalassaemia ≥12 months before enrolment and having received ≥6 red blood cell (RBC) units (excluding elective surgery) with no transfusion-free period ≥35 days in the 24 weeks before enrolment. Primary data were collected at a single visit and through chart review. Between Oct 21, 2019, and Jun 15, 2020, 201 eligible patients [median (interquartile range, IQR) age 45.7 (40.2-50.5) years; 75.6% > 40 years old; 64.2% female] were enrolled, a mean (standard deviation) of 42.9 (7.8) years after diagnosis. Median (IQR) age at diagnosis and RBC transfusion initiation were 0.8 (0.4-2.8) and 1.3 (1.0-5.0) years, respectively. From diagnosis to enrolment, patients had developed a median of six (range: 1-55) complications; 19.6% were grade ≥3. The most represented complications were endocrine/metabolic/nutrition disorders (91.5%), surgical/medical procedures (67.7%) and blood/lymphatic system disorders (64.7%). Real-world data generated by ULYSSES underscore the substantial complication burden of transfusion-dependent ß-thalassaemia patients, routinely managed in Greece.