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

Abstract

BackgroundHistorically, 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. MethodsThis 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. FindingsIn total, 8 018 patients (56·6% females; 4 538/8 018) 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% (5 323/7 605), 22% (1 671/7 605), and 8% (611/7 605) 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. InterpretationPatients must be closely monitored during their hospitalizations to intensify treatment and check treatment compliance. Innovative therapies are also required. FundingThe study was funded by Novartis.