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,

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

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