Comparison between Adult Patients with Sickle Cell Disease of Sub-Saharan African Origin Born in Metropolitan France and in Sub-Saharan Africa

Vasco Honsel; Djamal Khimoud; Brigitte Ranque; Lucile Offredo; Laure Joseph; Jacques Pouchot; Jean-Benoît Arlet

doi:10.3390/jcm8122173

Comparison between Adult Patients with Sickle Cell Disease of Sub-Saharan African Origin Born in Metropolitan France and in Sub-Saharan Africa

J Clin Med. 2019 Dec 9;8(12):2173. doi: 10.3390/jcm8122173.

Authors

Vasco Honsel¹, Djamal Khimoud¹, Brigitte Ranque^{1

2

3

4}, Lucile Offredo⁴, Laure Joseph⁵, Jacques Pouchot^{1

2}, Jean-Benoît Arlet^{1

2

3}

Affiliations

¹ Internal Medicine Department, Sickle Cell Referral Center, Georges Pompidou European Hospital, AP-HP, 75015 Paris, France.
² Paris Descartes Faculty of Medicine, Sorbonne Paris-Cité, 75006 Paris, France.
³ Laboratory of Excellence GR-Ex, 75015 Paris, France.
⁴ INSERM U970 PARCC Equipe 4 "Epidémiologie cardiovasculaire et mort subite", 75015 Paris, France.
⁵ Department of Biotherapy, Necker Hospital, AP-HP, 75015 Paris, France.

Abstract

Sickle cell disease (SCD) prevalence has increased rapidly in Europe as a result of an increase in the life expectancy of these patients and the arrival of SCD migrants from Africa. The aim of our study was to compare the phenotypes of adult patients born in Sub-Saharan Africa (SSA) who migrated to France with those of patients with the same origin who were born in France. This single-center observational study compared the demographic, clinical and biological characteristics of SCD adult patients of SSA origin who were born in France or SSA. Data were collected from computerized medical charts. Groups were compared using multivariate logistic regression with adjustment for age, gender and type of SCD. Of the 323 SCD patients followed in our center, 235 were enrolled, including 111 patients born in France and 124 patients born in SSA. SCD genotypes were balanced between groups. Patients born in Africa were older (median age 32.1 (24.4-39) vs. 25.6 (22.1-30.5) years, p < 0.001) and more often women (n = 75 (60.5%) vs. 48 (43.2%), p = 0.008). The median age at arrival in France was 18 years (13-23). The median height was lower among patients born in SSA (169 (163-175) vs. 174.5 cm (168-179), p < 0.001). Over their lifetimes, patients born in France had more acute chest syndromes (median number 2 (1-4) vs. 1 (0-3), p = 0.002), with the first episode occurring earlier (19 (11.6-22.3) vs. 24 (18.4-29.5) years, p < 0.007), and were admitted to intensive care units more often (53.3% vs. 34.9%, p = 0.006). This difference was more pronounced in the SS/Sβ0 population. Conversely, patients born in SSA had more skin ulcers (19.4% vs. 6.3%, p = 0.03). No significant differences were found in social and occupational insertion or other complications between the two groups. Patients born in SSA had a less severe disease phenotype regardless of their age than those born in France. This difference could be related to a survival bias occurring in Africa during childhood and migration to Europe that selected the least severe phenotypes.

Keywords: Sub-Saharan Africa; acute chest syndrome; migration; sickle cell disease.