Sickle cell disease (SCD) is a common genetic disorder leading to severe hematologic and cardiovascular complications. Increasing lifespan of patients is associated with increased prevalence of cardiovascular diseases, especially high blood pressure (BP). However, the clinical and epidemiological characteristics of high BP in these patients remain poorly defined. Notably, the usefulness of 24h-ambulatory blood pressure monitoring (ABPM) has not been assessed in adults, and specific cut-off for BP values are still debated.

To analyze office blood pressure and 24-h ABPM values in SCD adult patients.

A prospective pilot study with systematic ABPM readings of SCD adult patients from October 2017 to April 2018, conducted in the SCD reference center of Martinique. Consistent Hypertension was defined as office BP≥140/90mmHg and 24-h mean BP≥130/80mmHg. Masked hypertension was defined as office BP<140/90mmHg and 24-h mean BP≥130/80mmHg. High Office BP was defined as SBP between 130–139mmHg and DBP between 85-89mmHg. White coat hypertension was defined as office BP≥140/90mmHg and 24-h mean BP<130/80mmHg.

We enrolled twenty-one patients age 50.9 years (±14.3). Hypertension was diagnosed in 10 patients (47.6%) of which 7 (70% of the hypertensive, 33.3% of the entire group) had consistent hypertension and 3 had masked hypertension (30%, 14.3% respectively). All the patients with masked hypertension had high office BP. One patient (4.8%) was found to have white coat hypertension. In univariate analysis, age, weight and SC genotype were positively associated with presence of hypertension (all P<0.05).

This pilot study shows an unexpected high frequency of hypertension, involving nearly one out of two adult patients with Sickle Cell Disease. It underscores the high frequency of masked hypertension, and underlines the need of ABPM in the clinical management of patients with sickle cell disease.