Or register here
Global Search

Trusted Resources: Evidence & Education

Scientific literature and patient education texts

Back to Evidence & Education / Patient Education

PH and Sickle Cell Disease

key information

source: Pulmonary Hypertension Association

year: N/A

summary/abstract:

Sickle cell disease (SCD), a type of anemia (shortage of red blood cells), is characterized by abnormally shaped red blood cells. Sickle cells (named for the sickle, or oblong, slender shape of the malformed blood cells) have decreased flexibility, meaning they break easily. This results in a number of life-long complications.

PH is an increasingly recognized complication of SCD. Studies show that approximately 30% of screened adult patients with SCD develop mild PH in adulthood. In 10% of patients, the PH is more severe. Recent autopsy studies also suggest that up to 75% of sickle cell patients show changes in the lung tissue at the time of death, indicating the existence of pulmonary arterial hypertension (PAH).

One possible reason sickle cell patients seem to be at risk for developing PH is that increased breakdown of red blood cells within the blood vessels causes inflammation and a decrease of nitric oxide (a substance that causes relaxation of the blood vessels). This causes constriction of the small arteries of the lungs which may lead to PH.

read more

Related Content

To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information

The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.

Close

© 2025 rareLife solutions Inc.

To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information

The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.

Close