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The vaso‐occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management
Author(s) -
Darbari Deepika S.,
Sheehan Vivien A.,
Ballas Samir K.
Publication year - 2020
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.13430
Subject(s) - medicine , vaso occlusive crisis , pathophysiology , disease , hyperalgesia , intensive care medicine , sickle cell anemia , nociception , receptor
Early diagnosis, treatment, and prevention of a vaso‐occlusive crisis (VOC) are 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. The development of agents for prevention and treatment of VOCs should be driven by our understanding of its pathophysiology.