What is it about?
People with Sickle Cell Disease have fragile red blood cells that easily break and release a substance called heme into the blood. Heme is useful inside red blood cells, but when it is free, it can damage blood vessels, trigger inflammation, and contribute to serious complications. The body has natural defense systems to safely bind and clear heme, but when heme is constantly released from fragile red blood cells, these systems can become overwhelmed. As a result, some heme remains “unprotected” and free to cause harm. In this study, we developed new analytical methods that allow us to measure not only the amount of heme present in the blood but also to quantify how much is safely bound and how much remains in an unprotected form. Using these methods, we found that people with sickle cell disease can have similar total heme levels as healthy individuals, yet carry much higher amounts of unprotected, harmful heme. These unprotected forms of heme are linked to biological processes that drive complications in sickle cell disease.
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Photo by Nsey Benajah on Unsplash
Why is it important?
Distinguishing the different forms of heme improves our understanding of clinical risk in Sickle Cell Disease and supports the development of treatments that strengthen the body’s natural heme‑clearing defenses.
Perspectives
To me, this work highlights the exceptional collaboration across industry R&D, academic researchers, and clinical teams. A sincere thank you to the full project team and all study contributors for their dedication and teamwork.
Moritz Saxenhofer
CSL Behring
Hemopexin-accessible heme emerges as a correllate of biomarkers of endothelial and platelet activation previously implicated in the process of vaso-occlusion in sickle cell disease. This supports a hypothesis that hemopexin-accessible heme is a target for therapeutically administered hemopexin. This approach is currently being evaluated in a Phase 2 clinical trial. [NCT06699849]
Gregory Kato
CSL Ltd
Read the Original
This page is a summary of: Plasma heme pool compartmentalization is linked to pathophysiology in Sickle Cell Disease, PLOS One, March 2026, PLOS,
DOI: 10.1371/journal.pone.0343527.
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