Sickle cell disease (SCD) is a recessive hereditary condition. The physical changes caused by SCD affect the quality of life (QoL) by negatively impacting psychological aspects. This study aimed to assess the prevalence of depression and anxiety in SCD patients based on different sociodemographic characteristics in Jeddah, Saudi Arabia. A cross-sectional study was conducted at King Abdulaziz University Hospital (KAUH) in Jeddah from 13 July to 30 August 2021. The included patients were 18 years of age and above and affected with sickle cell disease. Medical staff interviewed the patients and filled the Patient Health Questionnaire (PHQ)-9 and General Anxiety Disorder (GAD)-7. One hundred nineteen (119) patients were included in this study. The median age of participants was 32 and mostly male (n=72, 60.5%). The rate of depression was 45.4%. On the other hand, the rate of anxiety was 22.7%. The median of the PHQ-9 score was 8±8 while the median of the GAD-7 score was 5±8. Moreover, the study showed that anxiety and depression in relation to sociodemographics were higher in the patient age groups of 30-34 years old, male, single, unemployed, and with higher education. There was a significant association between depression rate and the two variables: patient employment status (49.3%; p=0.047) and a family history of SCD (51%). Depression in patients with sickle cell disease is prevalent and correlated to demographic and social factors.

Background: In the era of the coronavirus disease-2019 (COVID-19) pandemic, the race toward shielding the public through vaccination is still going. Patients with sickle cell disease (SCD) require special consideration given their medical needs and the common side effects of immunization, affecting their decision. Therefore, we aimed to assess the perception and hesitancy toward COVID-19 vaccination in this population and explore the possible factors when it comes to vaccination decisions. Methods: The present cross-sectional phone interview study was conducted between May 10 and 20, 2021. The questionnaire was administered by the medical staff. The participants were all patients with SCD presented to King Abdulaziz University Hospital in Jeddah, Saudi Arabia. Results: Out of 346 patients, 147 patients agreed to participate. Only 52 (35.37%) patients received at least one dose of the nationally available vaccines, and there were no reported serious side effects. Among the unvaccinated participants, 45 patients (47.8%) were undecided. The most reported reasons for hesitancy were the fear of developing complications as their acquaintance had and the fear of developing brain blood clots post vaccination. Conclusions: The number of vaccinated patients with SCD was unfortunately low in our study, secondary to hesitancy. This represents a significant barrier and needs to be tackled appropriately at any proper interaction with a patient with SCD. The absence of major side effects and vaso-occlusive crises is assuring.

Introduction: Studies assessing immune responses following Pfizer-BioNTech BNT162b2 mRNA COVID-19 (Pfizer) and ChAdOx1 nCoV-19 AZD1222 (AstraZeneca) vaccines in patients with hemoglobinopathy are non-existent in the literature despite being thought at high risk of infection. Methods: Prospectively, we collected serum from patients with hemoglobinopathies at least 14 days post vaccine and measured neutralizing antibodies (nAb) in addition to binding antibodies using in-house assays. Results: All 66 participants mounted a significant binding antibody response (100%), but nAbs were detected in (56/66) post-vaccine with a rate of 84.5%. Age, gender, vaccine type, spleen status, hydroxyurea use, and hyperferritinemia did not affect the rate significantly. While 23/32 (71.8%) patients receiving only one dose of the vaccine were able to mount a positive response, 33/34 (97.05%) of those who had two doses of any vaccine type had a significant nAbs response. Patients who had anti-nucleocapsid (N), signifying asymptomatic infection in the past, were able to produce nAbs (31/31). No nAbs were detected in 10/35 (28.5%) patients with no anti-N antibodies. Conclusion: Our results provide supportive data when advising patients with hemoglobinopathy to receive COVID-19 vaccines and ensure booster doses are available for better immunity. Whenever available, measurement of nAb is recommended.

Key Clinical Message: Vincristine therapy can be effective in refractory Immune thrombocytopenia (ITP) following COVID‐19 vaccination. Our case report highlights the need for further research to establish standard management guidelines for COVID‐19‐vaccine‐associated ITP. Adult immune thrombocytopenia (ITP) can occur as a rare complication following several viral infections or a rare adverse event or complication of vaccination. In this paper, we report a case of a 39‐year‐old male patient with severe refractory ITP that began 4‐weeks after receiving his third (booster) dose of the COVID‐19 vaccine (BNT162b2, Pfizer‐BioNTech). He was given oral dexamethasone 40 mg daily for 4 days followed by prednisone at 1 mg/kg (85 mg daily) for 10 days. In the following weeks, we attempted several other lines of therapy to treat his ITP, including anti‐RhD immunoglobulin, which, unfortunately, caused moderate hemolysis requiring packed red blood cell transfusion, intravenous immunoglobulin (given at a subtherapeutic dose of 0.4 g/kg for only 1 day since it was not available), rituximab, and eltrombopag. The patient, unfortunately, showed no response to any of these treatments. This was an indicator to initiate salvage therapy with vincristine 2 mg weekly for 3 weeks. The patient's platelet count started to increase remarkably during the third week of vincristine and normalized after 4 weeks. We review the findings, clinical characteristics, and management approaches that were reported in the literature regarding COVID‐19‐vaccine‐induced ITP. More in‐depth research is needed to delineate standard guidelines for the management of such cases. This report underscores the importance of resorting to vincristine and eltrombopag as great options for severe and refractory ITP related to the COVID‐19 vaccine.

Sickle cell hepatopathy is an underreported entity lacking clear management guidelines. This case highlights the potential role of hydroxyurea (HU) in improving the hepatic dysfunction seen among patients with sickle cell disease (SCD). We herein present the clinical course of a patient prior to and after the initiation of hydroxyurea with an emphasis on long-term outcomes and the patterns of liver injury over a 15-year time course.

BACKGROUND: Thalassemia is a hereditary hemoglobinopathy characterized by inadequate or absent globin synthesis. This study aims to assess the prevalence and incidence of chronic pain in individuals with thalassemia within our population and further investigate the underlying risk factors and precipitating triggers. SUBJECTS AND METHODS: This retrospective cross-sectional study was conducted at King Abdulaziz University Hospital in Jeddah, KSA, from March to June 2023. Using an electronic data collection sheet, we contacted patients by phone and accessed hospital records. Regression results were presented as odds ratios (ORs) with 95% confidence intervals, and statistical significance was defined as a P < 0.05. RESULTS: Ninety-one thalassemia patients were enrolled with a mean age of 26.22 ± 14.64 years. Most patients, 82.9%, had beta-thalassemia major, and 50 patients, 54.9%, reported experiencing pain during the past 4 weeks. Among these patients, the most frequently affected pain site was the left leg. In addition, 88% of participants reported pain triggers, with physical exertion being the most common. Patients who were single (P = 0.035), those with previous hospital admissions to intensify chelation (P = 0.046), and those without symptoms of iron overload (P < 0.001) were significantly associated with increased chronic musculoskeletal pain. CONCLUSION: The occurrence of pain increases with aging and lower hemoglobin levels, leading to a significant deterioration in the quality of life for individuals with thalassemia. More than half of the patients experienced musculoskeletal discomfort in the preceding month. In total, 40% of the patients began experiencing pain within a year from the start of our study, while the remaining individuals had been enduring similarly distressing symptoms for over a year.