Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions

Kato, Gregory J.; Hebbel, Robert P.; Steinberg, Martin H.; Gladwin, Mark T.

doi:10.1002/ajh.21475

Cited by 349Hemoglobinopathies and Related DisordersBlood properties and coagulationHemoglobin structure and function

Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions

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Kato, Gregory J., Hebbel, Robert P., Steinberg, Martin H., Gladwin, Mark T.·2009·American Journal of Hematology

DOI 10.1002/ajh.21475PMC PMC3209715

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Abstract

Sickle cell disease has been very well characterized as a single amino acid molecular disorder of hemoglobin leading to its pathological polymerization, with resulting red cell rigidity that causes poor microvascular blood flow, with consequent tissue ischemia and infarction. More recently, an independent spectrum of pathophysiology of blood vessel function has been demonstrated, involving abnormal vascular tone and activated, adhesive endothelium. These vasculopathic abnormalities are attributable to pathways involving hemolysis-associated defects in nitric oxide bioavailability, oxidative stress, ischemia-reperfusion injury, hemostatic activation, leukocytes and platelets. Vasculopathy of sickle cell disease has been implicated in the development of pulmonary hypertension, stroke, leg ulceration and priapism, particularly associated with hemolytic severity, and reported also in other severe hemolytic disorders. This vasculopathy might also play a role in other chronic organ dysfunction in patients with sickle cell disease. These pathways present novel targets for pharmacologic intervention, and several clinical trials are already under way. The authors present their perspectives of a workshop held at the National Institutes of Health in August 2008 on vasculopathy in sickle cell disease, along with meritorious future scientific questions on the topic of vascular complications of sickle cell disease.

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There is a surprising similarity between the arteriopathy of the central nervous system and pulmonary arterial vasculature in sickle cell disease, and both are epidemiologically and mechanistically related to the severity of hemolytic anemia.
“There is a surprising similarity between the arteriopathy of the CNS and pulmonary arterial vasculature in SCD, and both are epidemiologically and mechanistically related to the severity of the hemolytic anemia (183).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [183]
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Authors: Kato, G. J., Hebbel, R. P., Steinberg, M. H., & Gladwin, M. T.
Affiliations: National Institutes of Health (US)
University of Minnesota (US)
Boston University (US)
University of Pittsburgh (US)
Corresponding author: Kato, Gregory J.
ORCID: Kato, Gregory J.
Hebbel, Robert P.
Steinberg, Martin H.
Gladwin, Mark T.
Venue: American Journal of Hematology
Publisher: Wiley
Published: 2009-08-25
Volume: 84
Issue: 9
Pages: 618-625
ISSN: 0361-8609 (ISSN-L)
1096-8652
DOI: 10.1002/ajh.21475
PMC: PMC3209715
OpenAlex: W2082679209
Cited by: 349
References: 151
Citations by year: 2026: 7 · 2025: 13 · 2024: 19 · 2023: 20 · 2022: 23 · 2021: 21 · 2020: 24 · 2019: 21 · 2018: 23 · 2017: 28 · 2016: 16 · 2015: 20 · 2014: 26 · 2013: 21 · 2012: 23
FWCI: 10.44
Type: journal article
Language: en
Open access: hybrid
doi.org
Licence: public-domain
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations: American Journal of Hematology (publishedVersion, public-domain)
PubMed (publishedVersion)
Europe PMC (PubMed Central) (acceptedVersion)
PubMed Central (acceptedVersion)
Funders: Ikaria
National Institutes of Health
National Heart, Lung, and Blood Institute (ZIAHL006012, ZIAHL006014)
NIH Clinical Center
Topics: Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Blood properties and coagulation
Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Hemoglobin structure and function
Keywords: sickle cell disease, vasculopathy, hemoglobin polymerization, red cell rigidity, microvascular blood flow, endothelial activation, nitric oxide bioavailability, oxidative stress, ischemia-reperfusion injury, hemostatic activation, leukocytes, platelets, pulmonary hypertension, stroke, leg ulcers, priapism, vascular complications, pharmacological interventions
MeSH terms: Anemia, Sickle Cell, Humans, Vascular Diseases

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Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions

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