source: American Society of Hematology

year: 2017

authors: Dapa Diallo, Mehdi Khellaf, Gonzalo De Luna, Sandrine Dautheville, Louis Affo, Stephanie Ngo, Tatiana besse-Hammer, Yvon Segbena, jean Benoit Arlet, François Lionnet, Sylvain le-Jeune, Anoosha Habibi, Bertrand Godeau, Frédéric Galactéros, Pablo Bartolucci

Introduction: Vaso-Occlusive Crisis (VOC), the most common manifestation of sickle cell disease (SCD), is the first cause of death, particularly when complicated by an acute chest syndrome (ACS). In the PRESEV1 study, we developed a predictive score of secondary ACS in SCD patient admitted in the hospital for VOC (AUC = 0.840 [95% CI: 0.780 – 0.900]), with a negative predictive value of 98.8% for the low level risk and a positive predictive value of 39.5%. The aim of the PRESEV-2 study is to validate the predictive score of ACS on adults and children with SCD in Europe and Africa. Herein we present preliminary data on 235 adults with a severe VOC included in 11 referral centers from 4 countries.

Patients and Methods: We conduct an international multicenter prospective observational study. The primary outcome measure is the occurrence of an ACS. The ACS is defined as a new auscultatory abnormality (crepitation or bronchial breathing) OR the association of a new radiologic infiltrate AND chest pain or decreased breath sounds. Secondary outcome measures are length of hospitalization, transfusion, morphine consumption, mortality and hospitalization in intensive care unit. Inclusion criteria are: SS or S-b0 thalassemia patients, VOC admitted at the emergency unit (VOC is defined as pain or tenderness affecting at least one part of the body not attributable to other causes) that required hospitalization and opioids (level 3). Exclusion criteria are: no inaugural ACS, homeless patients, pregnancy, deprived of their liberty or under guardianship, unable to understand the purpose and conditions of carrying out the study, unable to give consent.

Results: From march 2016 to june 2017, 112 adult patients were included in Africa and 122 adults in Europe. Patients in Africa were younger than in Europe with a mean age, respectively, of 26 ± 7 yrs and 29 ± 8 yrs (p=0.008). Sex ratio (M/F) in Africa was 0.84 and 0.95 in Europe. Use of hydroxyurea was significantly higher in Europe (65%) than in Africa (6%) (p<0.0001). The ACS incidence was significantly higher in Africa than in Europe, respectively 22% and 11% (OR=2.24; p=0.03), in both areas the time for ACS appearance was the second day. Transfusion was performed in 27% in Africa and 6% in Europe (p<0.001), ACS was the main cause of transfusion (53.3% in Africa and 62.5% in Europe). Patients with ACS were more transfused in Africa than in Europe, respectively 64% and 35.7% but the difference was not significant. Mortality was 3.6% in Africa and was not observed in Europe; circumstances of death were stroke (1), profound anemia (1), kidney failure (1) and VOC (1).

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