Sickle cell disease is associated with chronic activation of the coagulation system. This may be associated with an increased risk of thromboembolic disease. A thromboembolic event may be the first indication of sickle cell disease.
The risk of thromboembolic disease can be found in all forms of sickle cell disease including sickle cell trait. It may require one or more risk factors to be manifest.
Factors that can trigger thromboembolism in a patient wth sickle cell hemoglobin:
(1) hyperviscosity
(2) an intravascular catheter
(3) extreme exercise and/or dehydration
(4) high-altitude exposure
Differential diagnosis:
(1) other hereditary hypercoagulable disorder
(2) acquired hypercoagulable state
A patient with sickle cell disease should be asked about any previous episodes of thromboembolism.
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