Anticoagulation For Pulmonary Hypertension in Sickle Cell Disease
Status:
Terminated
Trial end date:
2012-09-01
Target enrollment:
Participant gender:
Summary
Sickle cell disease (SCD) is often referred to as a hypercoagulable state. However, the
contribution of coagulation activation to the pathogenesis of SCD remains uncertain.
Pulmonary hypertension (PHT) is a common complication associated with significant morbidity
and mortality. Autopsy studies of SCD patients with PHT show evidence of in situ thrombosis
involving pulmonary vessels, similar to findings in non-sickle cell patients with PHT.
Anticoagulation has been reported to be of benefit in non-sickle cell patients with PHT. With
the evidence of increased coagulation activation in SCD, PHT represents a clinical endpoint
that may be used to evaluate the contribution of coagulation activation to the
pathophysiology of SCD. The investigators hypothesize that increased thrombin generation, as
well as platelet activation are central to the pathophysiology of SCD and contribute to the
occurrence of several SCD-related complications, including PHT. As a consequence, treatment
modalities that down-regulate thrombin generation would be expected to delay the progression
of PHT and result in improved survival in patients with SCD.