Trusted Resources: Evidence & Education
Scientific literature and patient education texts
The Vaso-Occlusive Pain Crisis in Sickle Cell Disease: Definition, Pathophysiology, and Management
source: European Journal of Haematology
authors: Deepika S. Darbari, Vivien A. Sheehan, Samir K. Ballassummary/abstract:
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
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