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.
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Department of Medicine, Howard University College of Medicine, Washington, DC, USA.
D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology; D004620:Embolism, Fat / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D015995:Prevalence