EHA Library - The official digital education library of European Hematology Association (EHA)

ASSOCIATION BETWEEN HOSPITALISED VASO-OCCLUSIVE CRISES AND MORTALITY IN SICKLE CELL DISEASE PATIENTS AGED 16 YEARS AND OLDER USING THE FRENCH NATIONAL HEALTH INSURANCE DATABASE (SNDS)
Author(s): ,
Jean-Benoît Arlet
Affiliations:
Médecine interne,Hôpital Européen Georges-Pompidou,Paris,France
,
Hannah Lennon
Affiliations:
Méthodes et Statistiques,HEVA,Lyon,France
,
Miranda Bailey
Affiliations:
Gene therapies,Novartis,Bannockburn, IL,United States
,
Eleonore Herquelot
Affiliations:
Méthodes et Statistiques,HEVA,Lyon,France
,
Ludovic Lamarsalle
Affiliations:
HEVA,Lyon,France
,
Fanny Raguideau
Affiliations:
Méthodes et Statistiques,HEVA,Lyon,France
Pablo Bartolucci
Affiliations:
Hopitâl Henri-Mondor,Créteil,France
EHA Library. Arlet J. 06/09/21; 324928; EP1207
Jean-Benoît Arlet
Jean-Benoît Arlet
Contributions
Abstract
Presentation during EHA2021: All e-poster presentations will be made available as of Friday, June 11, 2021 (09:00 CEST) and will be accessible for on-demand viewing until August 15, 2021 on the Virtual Congress platform.

Abstract: EP1207

Type: E-Poster Presentation

Session title: Sickle cell disease

Background
Patients with sickle cell disease (SCD) experience a wide range of complications thought to be due to the systemic impact of chronically inflamed vasculature, ongoing haemolysis, multi-cellular adhesion and ischemic damage. One of the most impactful complications is the vaso-occlusive crisis (VOC). An early study suggested a relationship between VOC occurrence and increased mortality. Reviewing this possible relationship, now that SCD management has improved, is important.

Aims
To assess the association between hospitalised VOCs and mortality, in SCD patients.

Methods
This study was a retrospective observational cohort study using the French national health insurance’s claims database. This database gathers information on hospital records, primary and secondary care, and cause of death. Between 01-01-2012 and 12-31-2018, all 16 years and older patients identified as suffering from SCD (with either a hospital record or Long-Term Disease status with diagnostic ICD-10 codes D57.0-2) were included in the study. The index date was defined as the first interaction with the healthcare system (whether it was related to their SCD or not). Participants were followed-up for at least one year and until the end of the study (12-31-2018).

A hospitalised VOC was defined as a hospital stay of at least one night with a primary or related diagnosis of sickle-cell anaemia with crisis (D57.0), preceded by a transit through the emergency room (to avoid capturing SCD-related hospital stays for reasons other than VOCs).


The hospitalized VOC annual rate was defined as the total number of hospitalized VOCs during the follow-up divided by the number of follow-up years. Patients were categorised by the annual rate of VOCs they experienced into three groups: <1; 1 to <3; ≥3 VOCs per year.


Cox proportional hazards models using age as the time scale were used to study the effect of the cumulative rate of hospitalised VOCs on the risk of death, adjusted for gender.

Results

A total of 17,726 SCD patients were included in the study, of which 65.3% were females —a high proportion partly due to several hospitalizations found in women while pregnant. Ages ranged from 16 to 99 years and the average age was 35.0 years (±17.8, median 32.0).


Each calendar year, 86–89%, 9–10%, and 2–4% of patients experienced <1, 1 to <3, and ≥3 hospitalised VOCs, respectively. The mean age in increasing VOC groups was 36.3 (±15.8), 23.8 (±9.0), and 21.3 years old (±7.1), respectively. The proportion of women was 66.7%, 51.8% and 52.9%, respectively. The median time between two hospitalised VOCs was 37 days (Q1–Q3 22–96).


Over a median follow up of 7 years, 914 (5.2%) patients died; 75% died at the hospital. The mean age at death was 60.1 years (±19.7).


Patients with an average of ≥3 hospitalised VOCs per year over the follow-up were 4.73 times [2.95;7.61] more at risk of death during the follow-up period than patients with <1 hospitalised VOC per year.

Conclusion
Our study confirms, in a nationwide cohort in France in a real-life setting, that patients frequently experiencing hospitalised VOCs are at higher risk of death compared to patients seldom experiencing VOCs. The highest risk of hospitalized VOC recurrence was within 100 days of a prior hospitalized VOC. Therefore, patients with recurring VOCs are at greatest need for innovative therapies that could reduce or prevent these events. Patients who are able to reduce their annual number of VOCs may live longer.

Keyword(s): Complications, Epidemiology, Mortality, Sickle cell disease

Presentation during EHA2021: All e-poster presentations will be made available as of Friday, June 11, 2021 (09:00 CEST) and will be accessible for on-demand viewing until August 15, 2021 on the Virtual Congress platform.

Abstract: EP1207

Type: E-Poster Presentation

Session title: Sickle cell disease

Background
Patients with sickle cell disease (SCD) experience a wide range of complications thought to be due to the systemic impact of chronically inflamed vasculature, ongoing haemolysis, multi-cellular adhesion and ischemic damage. One of the most impactful complications is the vaso-occlusive crisis (VOC). An early study suggested a relationship between VOC occurrence and increased mortality. Reviewing this possible relationship, now that SCD management has improved, is important.

Aims
To assess the association between hospitalised VOCs and mortality, in SCD patients.

Methods
This study was a retrospective observational cohort study using the French national health insurance’s claims database. This database gathers information on hospital records, primary and secondary care, and cause of death. Between 01-01-2012 and 12-31-2018, all 16 years and older patients identified as suffering from SCD (with either a hospital record or Long-Term Disease status with diagnostic ICD-10 codes D57.0-2) were included in the study. The index date was defined as the first interaction with the healthcare system (whether it was related to their SCD or not). Participants were followed-up for at least one year and until the end of the study (12-31-2018).

A hospitalised VOC was defined as a hospital stay of at least one night with a primary or related diagnosis of sickle-cell anaemia with crisis (D57.0), preceded by a transit through the emergency room (to avoid capturing SCD-related hospital stays for reasons other than VOCs).


The hospitalized VOC annual rate was defined as the total number of hospitalized VOCs during the follow-up divided by the number of follow-up years. Patients were categorised by the annual rate of VOCs they experienced into three groups: <1; 1 to <3; ≥3 VOCs per year.


Cox proportional hazards models using age as the time scale were used to study the effect of the cumulative rate of hospitalised VOCs on the risk of death, adjusted for gender.

Results

A total of 17,726 SCD patients were included in the study, of which 65.3% were females —a high proportion partly due to several hospitalizations found in women while pregnant. Ages ranged from 16 to 99 years and the average age was 35.0 years (±17.8, median 32.0).


Each calendar year, 86–89%, 9–10%, and 2–4% of patients experienced <1, 1 to <3, and ≥3 hospitalised VOCs, respectively. The mean age in increasing VOC groups was 36.3 (±15.8), 23.8 (±9.0), and 21.3 years old (±7.1), respectively. The proportion of women was 66.7%, 51.8% and 52.9%, respectively. The median time between two hospitalised VOCs was 37 days (Q1–Q3 22–96).


Over a median follow up of 7 years, 914 (5.2%) patients died; 75% died at the hospital. The mean age at death was 60.1 years (±19.7).


Patients with an average of ≥3 hospitalised VOCs per year over the follow-up were 4.73 times [2.95;7.61] more at risk of death during the follow-up period than patients with <1 hospitalised VOC per year.

Conclusion
Our study confirms, in a nationwide cohort in France in a real-life setting, that patients frequently experiencing hospitalised VOCs are at higher risk of death compared to patients seldom experiencing VOCs. The highest risk of hospitalized VOC recurrence was within 100 days of a prior hospitalized VOC. Therefore, patients with recurring VOCs are at greatest need for innovative therapies that could reduce or prevent these events. Patients who are able to reduce their annual number of VOCs may live longer.

Keyword(s): Complications, Epidemiology, Mortality, Sickle cell disease

By clicking “Accept Terms & all Cookies” or by continuing to browse, you agree to the storing of third-party cookies on your device to enhance your user experience and agree to the user terms and conditions of this learning management system (LMS).

Cookie Settings
Accept Terms & all Cookies