Neutrophils in sickle cell disease: Exploring their potential role as a therapeutic target.

Factors influencing the activation of neutrophils in SCD and the potential neutrophil-mediated ameliorating effects of therapies in SCD.

Fluid overload due to intravenous fluid therapy for vaso-occlusive crisis in sickle cell disease: incidence and risk factors.

Intravenous fluid therapy (IV-FT) is routinely used in the treatment of vaso-occlusive crises (VOCs), as dehydration possibly promotes and sustains erythrocyte sickling. Patients with sickle cell disease (SCD) are at risk of developing diastolic dysfunction and fluid overload due to IV-FT. However, data on the adverse effects of IV-FT for VOC is sparse. We aimed to evaluate the incidence and risk factors of fluid overload due to IV-FT in patients with SCD. Consecutive hospitalisations for VOC treated with IV-FT between September 2016 and September 2018 were retrospectively analysed. The median (interquartile range) age was 25·0 (18·3-33·8) years and 65% had a severe genotype (HbSS/HbSß0 -thal). Fluid overload occurred in 21% of 100 patients. Hospital stay was longer in patients with fluid overload (6·0 vs. 4·0 days, P = 0·037). A positive history of fluid overload (P = 0·017), lactate dehydrogenase level (P = 0·011), and top-up transfusion during admission (P = 0·005) were independently associated with fluid overload occurrence. IV-FT was not reduced in 86% of patients despite a previous history of fluid overload. Fluid overload is frequently encountered during IV-FT for VOC. IV-FT is often not adjusted despite a positive history of fluid overload or when top-up transfusion is indicated, emphasising the need for more awareness of this complication and a personalised approach.

Association Between Anemia and Cerebral Venous Thrombosis: Case-Control Study.

BACKGROUND AND PURPOSE: Anemia is often considered to be a risk factor for cerebral venous thrombosis (CVT), but this assumption is mostly based on case reports. We investigated the association between anemia and CVT in a controlled study. METHODS: Unmatched case-control study: cases were adult patients with CVT included in a single-center, prospective database between July 2006 and December 2014. Controls were subjects from the control population of the Multiple Environmental and Genetic Assessment of Risk Factors for Venous Thrombosis (MEGA) study. Anemia was defined according to World Health Organization criteria: nonpregnant women hemoglobin<7.5 mmol/L, pregnant women<6.9 mmol/L, and men<8.1 mmol/L. We used logistic regression analysis, adjusting for age, sex, malignancy, oral contraceptive use, and pregnancy/puerperium. RESULTS: We included 152 cases and 2916 controls. Patients with CVT were younger (mean age, 40 versus 48 years) and more often women (74% versus 53%) than controls. Anemia was more frequent in cases (27.0%) than in controls (6.5%; P<0.001). Anemia was associated with CVT, both in univariate analysis (odds ratio, 5.3; 95% confidence interval [CI], 3.6-7.9) and after adjustment for potential confounders (adjusted odds ratio, 4.4; 95% CI, 2.8-6.9). Hemoglobin as a continuous variable was inversely associated with CVT (adjusted odds ratio per 1 mmol/L change 0.53; 95% CI, 0.42-0.66). Stratification by sex showed a stronger association between anemia and CVT in men (adjusted odds ratio, 9.9; 95% CI, 4.1-23.8) than in women (3.6; 95% CI, 2.1-6.0). CONCLUSION: Our data suggest that anemia is a risk factor for CVT.