Systemic fat embolism and pulmonary hypertension in sickle cell disease
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.
Castro, O., "Systemic fat embolism and pulmonary hypertension in sickle cell disease" (1996). The Center For Sickle Cell Disease Faculty Publications. 255.