Sickle cell disease is associated with iron mediated hypercoagulability.

Area of Science:

• Hematology
• Coagulation Science
• Iron Metabolism

Background:

• Sickle cell disease (SCD) is characterized by a hypercoagulable state and hemostatic anomalies.
• Iron overload, a complication of transfusions in SCD patients, can enhance thrombin's action on fibrinogen, promoting rapid clot formation.
• The interplay between iron and coagulation in SCD requires further investigation.

Purpose of the Study:

• To investigate the hypothesis that SCD patients exhibit hypercoagulable plasma coagulation kinetics.
• To assess the potential for iron to enhance coagulation in SCD patients.
• To correlate viscoelastic coagulation data with clinical manifestations of SCD.

Main Methods:

• Viscoelastic methods were employed to assess coagulation kinetics and iron enhancement.
• Archived, citrated plasma samples from 20 SCD patients (ambulatory or hospitalized) were analyzed.
• Institutional Review Board (IRB) approval was obtained prior to sample analysis.

Main Results:

• All analyzed SCD patient plasma samples demonstrated hypercoagulability.
• A significant proportion (65%) of SCD patients showed evidence of iron enhancement of coagulation.
• Viscoelastic testing revealed accelerated thrombin generation and clot formation in the presence of iron.

Conclusions:

• SCD plasma exhibits inherent hypercoagulability.
• Iron plays a significant role in enhancing coagulation in a majority of SCD patients.
• Further research correlating viscoelastic data with clinical symptoms is warranted to understand iron's role in SCD complications like vaso-occlusive crisis.