How can the incidence of vaso-occlusive crisis in patients with sickle cell disease (SCD) be reduced?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

The randomized BABY HUG study has demonstrated that hydroxyurea (hydroxycarbamide) significantly reduces the incidence of vaso-occlusive crisis and dactylitis in very young children. [84] The primary toxicity observed was neutropenia. Further study is needed to evaluate long-term treatment effects on growth and development as well as renal, lung, and CNS function. A randomized, placebo-controlled trial in adults did not demonstrate a significant improvement in the time to resolution of vaso-occlusive crisis. [85]

Crizanlizumab, a P-selectin inhibitor, was approved by the FDA in November 2019 to reduce frequency of vaso-occlusive crisis (VOC) in adults with sickle cell disease. Binding P-selectin on the surface of activated endothelium and platelet cells blocks interactions between endothelial cells, platelets, red blood cells, and leukocytes. Approval was based on the SUSTAIN clinical trial which showed crizanlizumab reduced the median annual rate of VOCs leading to health care visits by 45.3% compared with placebo (1.63 vs 2.98, P=0.010) in patients with or without hydroxyurea. [61, 62]  


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