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scientific articles

The Vaso-Occlusive Pain Crisis in Sickle Cell Disease: Definition, Pathophysiology, and Management

key information

source: European Journal of Haematology

year: 2020

authors: Deepika S. Darbari, Vivien A. Sheehan, Samir K. Ballas


Early diagnosis, treatment, and prevention of a vaso-occlusive crisis (VOC) is critical to the management of patients with sickle cell disease. It is essential to differentiate between VOC-associated pain and chronic pain, hyperalgesia, neuropathy, and neuropathic pain. The pathophysiology of VOCs includes polymerization of abnormal sickle hemoglobin,inflammation, and adhesion.

Hydroxyurea, L-glutamine, crizanlizumab, and voxelotor have been approved by the US Food and Drug Administration for reducing the frequency of VOCs; the European Medicines Agency has approved only hydroxyurea. Other novel treatments are in late-stage clinical development in both the United States and the European Union. Development of agents for prevention and treatment of VOCs should be driven by our understanding of its pathophysiology.

organization: Children’s National Medical Center, USA; Baylor College of Medicine, USA; Thomas Jefferson University, USA

DOI: 10.1111/ejh.13430

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