Abstract Number: PB0195
Meeting: ISTH 2020 Congress
Theme: Coagulation and Natural Anticoagulants » Coagulation Factors and Inhibitors
Background: Sickle cell disease (SCD) is a chronic hereditary disease caused by a mutation in the hemoglobin gene. Apart from acute vaso-occlusive and anemic crisis, it is considered as a real pro-thrombotic state. One of the main risk factors of thrombosis is protein S deficiency, which is frequently observed during SCD.
In this context of chronic inflammation, the interpretation of its decline can be difficult.
Aims: We led a study to know the impact of this proinflammatory state in the PS deficiency observed during sickle cell anemia.
Methods: We included 50 homozygous and 50 heterozygous sickle cell patients. The mean age was 26 years and 20% of the SS had already had a thrombotic accident such as stroke and low limb thrombosis.
Results: The measurement of fibrinogen, as a marker of inflammation, showed hyperfibrinogenemia in 16% of the SS and 10% of the AS. The protein level was significantly lower in the SS than in the AS with 42% and 80% respectively. The prevalence of PS deficiency was thus 52% (SS) and 14% (AS). A weak correlation was found between hyperfibrinemia and protein S deficiency among sickle cell patients: R = 0.068.
We can thus say that despite the chronic inflammation state in sickle cell patients linked to illness or other intercurrent disease, protein S deficiency is indeed a risk factor for thrombosis in sickle cell patients, especially homozygous.
Conclusions: In addition to other hematological factors incriminated in the occurrence of thrombotic accidents in sickle cell anemia (APL, PC deficiency) the deficit in PS must be taken into account in the management of sickle cell anemia.
To cite this abstract in AMA style:
Touré AO, Seck M, Sall A, Sall FB, Coly MN, Faye BF, Gadji M, Diop S. Protein S Deficiency, Inflammation and Sickle Cell Anemia [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/protein-s-deficiency-inflammation-and-sickle-cell-anemia/. Accessed March 21, 2024.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/protein-s-deficiency-inflammation-and-sickle-cell-anemia/