Chronic osteo-articular changes in patients with sickle cell disease

Abstract

Background Sickle cell disease (SCD) is an autosomal recessive genetic disease in which a mutation occurs in the β-globin chain gene, resulting in abnormal hemoglobin levels. In an environment with reduced oxygen concentration, red blood cells change their conformation, resulting in chronic hemolysis and consequent anemia and vaso-occlusive crises with injuries to several organs, with a significant impairment of the osteoarticular system. This study aimed to verify the chronic osteoarticular alterations and their association with clinical and laboratory characteristics of patients with SCD with a more severe phenotype (SS and Sβ0), on a steady-state fasis. Methods Fifty-five patients were referred to a medical consultation with a specialized assessment of the locomotor system, followed by laboratory tests and radiographic examinations. Results In total, 74.5% patients had hemoglobinopathy SS; 67.3% were female; and 78.2% were non-whites. The mean patient age was 30.5 years. Most patients (61.8%) reported up to three crises per year, with a predominance of high-intensity pain (65.5%). Radiographic alterations were present in 80% patients. A total of 140 lesions were identified, most which were located in the spine, femur, and shoulders. Most lesions were osteonecrosis and osteoarthritis and were statistically associated with the non-use of hydroxyurea. Conclusions There was a high prevalence of chronic osteoarticular alterations, which was statistically associated only with the non-regular use of hydroxyurea.

Hip and Knee Osteoarthritis in Patients with Chronic Myeloproliferative Neoplasms: A Cross-Sectional Study

“…This important observation suggests that adequate control of the MPN clone with cytoreductive treatment may also have favorable effects on the osteoarticular system. It should also be pointed out that the majority of MPN patients included in the study were treated with HU; a similar protective effect of HU on the osteoarticular system was also found in patients with sickle cell anemia [ 64 ]. Considering that early therapeutic intervention (especially with interferons) in MPN has recently been put into limelight [ 65 , 66 ], future prospective studies are needed to investigate whether other cytoreductive treatments may also possess similar activity, and whether their early initiation in MPN may postpone OA development and progression.…”

Sickle Cell Disease Diagnosis Uncovered by Incidental Radiograph Findings

“…As hydroxyurea reduces SCD complications and improves survival, it is widely recommended for children, especially in the most clinically severe genotypes. Although robust studies on its effect on bone lesions are lacking, considering its mechanism in the microcirculation, a positive effect is expected in its prevention or progress 6…”

“…Although robust studies on its effect on bone lesions are lacking, considering its mechanism in the microcirculation, a positive effect is expected in its prevention or progress. 6…”

Diagnostic evaluation and treatment of osteoarticular lesions in patients having sickle cell anemia: Literature review

“…In a recent study, in adults with more severe phenotypes (SS and Sβº), most osteoarticular complications were identified as osteonecrosis and osteoarthritis, with the majority of patients experiencing up to three crises per year, with severe pain and radiographic changes in 80% of patients, especially related to lesions in the spine, femur, and shoulders, and associated with non-use of hydroxyurea (Ferreira et al, 2021).…”