Deadly Lung Disorder in Sickle Cell Disease

High blood pressure in the lungs is a major risk factor for death in sickle cell disease. Almost one-third of adults with sickle cell have pulmonary hypertension and those with pulmonary hypertension have a much higher mortality rate than those without pulmonary hypertension.

Comments: Pulmonary hypertension develops in most genetic diseases with chronic hemolysis (breakup of red blood cells) and anemia, including sickle cell disease, thalassemia, and hereditary spherocytosis. Everyone with these diseases should be screened for pulmonary hypertension. The screening can be done by a noninvasive method called Doppler echocardiography.

Barbara K. Hecht, Ph.D.
Frederick Hecht, M.D.
Medical Editors, MedicineNet.com

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High Blood Pressure in the Lungs a Major Risk for Death in Adults with Sickle Cell Disease

A new study reveals that nearly one third of adults with sickle cell disease develop high blood pressure in their lungs and that the condition, known as pulmonary hypertension, causes a much higher death rate in patients with the complication than those without it. The findings, according to researchers from the Warren Grant Magnuson Clinical Center at the National Institutes of Health (NIH), demonstrate an urgent need to diagnose this complication in adults with sickle cell disease as it is a major risk factor for death. The study was conducted as a multi-center collaboration between NIH and the Howard University Center for Sickle Cell Disease. A complete report will publish in the February 26 edition of the New England Journal of Medicine.