[French guidelines for the management of adult sickle cell disease: 2015 update].

[Article in French]

Habibi A1, Arlet JB2, Stankovic K3, Gellen-Dautremer J4, Ribeil JA5, Bartolucci P6, Lionnet F7; centre de référence maladies rares « syndromes drépanocytaires majeurs ».

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Abstract

Sickle cell disease is a systemic genetic disorder, causing many functional and tissular modifications. As the prevalence of patients with sickle cell disease increases gradually in France, every physician can be potentially involved in the care of these patients. Complications of sickle cell disease can be acute and chronic. Pain is the main symptom and should be treated quickly and aggressively. In order to reduce the fatality rate associated with acute chest syndrome, it must be detected and treated early. Chronic complications are one of the main concerns in adults and should be identified as early as possible in order to prevent end organ damage. Many organs can be involved, including bones, kidneys, eyes, lungs, etc. The indications for a specific treatment (blood transfusion or hydroxyurea) should be regularly discussed. Coordinated health care should be carefully organized to allow a regular follow-up near the living place and access to specialized departments. We present in this article the French guidelines for the sickle cell disease management in adulthood.

KEYWORDS:

Acute chest syndrome; Blood transfusion; Crise vaso-occlusive; Drépanocytose; Hydroxyurea; Hydroxyurée; Sickle cell disease; Syndrome thoracique aigu; Transfusion sanguine; Vaso-occlusive crisis