Systemic fat embolism and pulmonary hypertension in sickle cell disease

Abstract

Systemic fat embolism, a relatively rare complication of sickle cell disease, is difficult to diagnose and it is often fatal. A high index of suspicion and early transfusion therapy may provide the best chance for recovery. Sickle cell-related pulmonary hypertension can be documented by cardiac catheterization but has no proven treatment. Patients with this complication are usually adults, have a poor prognosis, and may be considered for hydroxyurea treatment. Administration of vasodilators, anticoagulation, or oxygen may be beneficial in selected individuals.