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Role of the coagulation system in the pathogenesis of sickle cell disease

Md Nasimuzzaman1,2, Punam Malik1,2

  • 1Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; and.

Blood Advances
|October 25, 2019
PubMed

Insights

Sickle cell disease (SCD) involves blood cell dysfunction and organ damage. Reducing thrombin (factor IIa) in mice improved survival and reduced inflammation and organ damage, highlighting coagulation

Area of Science:

  • Hematology
  • Vascular Biology
  • Thrombosis and Hemostasis

Background:

  • Sickle cell disease (SCD) is a genetic red blood cell disorder causing widespread organ damage.
  • Coagulation system activation and chronic inflammation are key features of SCD pathophysiology.
  • Thrombin (factor IIa) plays a central role in both hemostasis and inflammation in SCD.

Purpose of the Study:

  • To review the role of coagulation system activation in sickle cell disease pathophysiology.
  • To discuss the impact of thrombin and related factors on SCD complications.

Main Methods:

  • This is a review discussing existing literature on coagulation in SCD.
  • Studies in mouse models of SCD (Berkeley sickle mice) are referenced.

Main Results:

  • Pharmacologic or genetic reduction of prothrombin in sickle mice improved survival.
  • Reduced prothrombin levels ameliorated vascular inflammation and decreased end-organ damage in mice.
  • Factors upstream and downstream of thrombin are implicated in SCD pathogenesis.

Conclusions:

  • Coagulation system activation is a critical driver of SCD pathophysiology.
  • Targeting thrombin and related coagulation factors presents a potential therapeutic strategy for SCD.
  • Further research into coagulation pathways may reveal novel treatments for SCD complications.

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