Hemostatic abnormalities in sickle cell disease.

PURPOSE OF REVIEW: Although it has long been recognized that sickle cell disease (SCD) and other hemoglobinopathies are associated with a state of chronic hyperactivation of coagulation, the study of the epidemiology of venous thromboembolic (VTE) complications in SCD is only now beginning to evolve. In parallel, mechanistic studies of the hypercoagulable state in humans and mouse models implicate an increasingly important causative role of hemolysis.

RECENT FINDINGS: The case for SCD as a thrombophilic state has been strengthened by the recent literature. In an attempt to better understand the underlying mechanism(s), global assays of coagulation (thromboelastography and thrombin generation assays) have been utilized by several groups, but thus far, the results have been inconsistent, probably because of the technical differences. However, global assays continue to support the case for an important role of peripheral blood cells and their derived microparticles in promoting coagulation activation.

SUMMARY: VTE is an underappreciated and potentially morbid complication of SCD. The mechanisms underlying this hypercoagulable state are complex. A greater understanding of these pathways may lead to the rational selection of therapies that not only prevent thrombosis, but also impact on many of the other vaso-occlusive complications of SCD.