ArticleThe Lancet regional health. Europe2024
Impact of hospitalized vaso-occlusive crises in the previous calendar year on mortality and complications in adults with sickle cell disease: a French population-based study.
Article in The Lancet regional health. Europe, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed.
- Article
- Effectiveness of preimplantation genetic testing in sickle cell disease: insights from a single-center experience.Journal of assisted reproduction and genetics · 2026Article
- The Economic and Clinical Impact of Recurring Automated Red Blood Cell Exchange to Manage Sickle Cell Disease in the UK.PharmacoEconomics - open · 2025Article
- Genetic modifiers of frequent vaso-occlusive hospitalizations among individuals with sickle cell disease (SCD).Annals of hematology · 2025Article
- A novel red blood cell deformability biomarker is associated with hemolysis and vaso-occlusive crises in sickle cell disease.Scientific reports · 2025Article
- Novel clinical care models for patients with sickle cell disease.Hematology. American Society of Hematology. Education Program · 2024Review
- Inpatient Management of Pain Episodes in Children with Sickle Cell Disease: A Review.Children (Basel, Switzerland) · 2024Review
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5 authors.
Funding
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Abstract
Background: Historically, sickle cell disease (SCD) patients experiencing frequent hospitalized vaso-occlusive crises (HVOC) have been associated with increased mortality, yet recent data reflecting the widespread use of hydroxyurea and advancements in disease management remain limited. Our study aims to assess the association between HVOC and mortality or severe complications in patients with SCD in this new treatment landscape. Methods: This was a retrospective observational cohort study using the French national health data system. Between 01-01-2012 and 12-31-2018, all SCD patients ≥16 years old (ICD-10 codes D57.0-2) were included and followed until 12-31-2018. HVOC was defined as a hospitalization of ≥1 night with primary diagnosis of SCD with crisis, following an emergency room visit. The association between HVOC and severe complications was assessed with a Cox proportional hazards model. Findings: In total, 8018 patients (56.6% females; 4538/8018) were included. The 2018 SCD standardized one-year period prevalence was 17.9 cases/100,000 person-years [17.4; 18.3]. The mean rate was 0.84 (1.88) HVOC/person-year. In 2018, 70% (5323/7605), 22% (1671/7605), and 8% (611/7605) of patients experienced 0, 1-2, or 3+ HVOCs, respectively. The median survival time between HVOCs was 415 days [386; 439]. Overall, 312 patients died (3.9%) with a mean age of 49.8 (19.4). Compared to patients without HVOC, the hazard ratios of death in patients with 1-2 or 3+ HVOCs the year prior to death were 1.67 [1.21; 2.30] and 3.70 [2.30; 5.93], respectively. Incidence of acute chest syndrome, pulmonary embolism, osteonecrosis, and sepsis increased with the HVOCs category, but not stroke. In 2018, 29.5% (180/611) of patients with 3+ HVOCs did not take hydroxyurea. Interpretation: Patients must be closely monitored during their hospitalizations to intensify treatment and check treatment compliance. Innovative therapies are also required. Funding: The study was funded by Novartis.
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