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Review
. 2020 Sep;105(3):237-246.
doi: 10.1111/ejh.13430. Epub 2020 May 19.

The vaso-occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management

Deepika S Darbari  1 , Vivien A Sheehan  2 , Samir K Ballas  3
Affiliations
Review

The vaso-occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management

Deepika S Darbari et al. Eur J Haematol. 2020 Sep.

Abstract

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.

Keywords: L-glutamine; P-selectin; hydroxyurea; pain; sickle cell disease.

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References

REFERENCES

    1. Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions. PLoS Medicine. 2013;10:e1001484.
    1. Lanzkron S, Carroll CP, Haywood C Jr. The burden of emergency department use for sickle-cell disease: an analysis of the national emergency department sample database. Am J Hematol. 2010;85:797-799.
    1. Ballas SK, Lusardi M. Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance. Am J Hematol. 2005;79:17-25.
    1. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med. 1994;330:1639-1644.
    1. Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a critical reappraisal. Blood. 2012;120:3647-3656.