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39 - Sickle Cell Emergencies

from Section 8 - Hematology–Oncology Emergencies

Published online by Cambridge University Press:  02 November 2023

Kaushal Shah
Affiliation:
Weill Cornell Medical Center, New York
Jarone Lee
Affiliation:
Massachusetts General Hospital, Boston
Clark G. Owyang
Affiliation:
Weill Cornell Medical Center, New York
Benjamin Christian Renne
Affiliation:
Massachusetts General Hospital, Boston
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Summary

  • Sickle cell disease (SCD) is caused by an autosomal recessive mutation in the β-globin chain of hemoglobin A (HbA), causing the mutated hemoglobin S (HbS). In a vaso-occlusive crisis (VOC), sickled red blood cells (RBCs) increase the viscosity of blood and cause microvascular occlusion, contributing to hypoxia, acidosis and further sickling. The clinical manifestations of SCD are diverse and are related to chronic hemolysis with periodic episodes of vascular occlusion that can affect nearly every organ system.

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Publisher: Cambridge University Press
Print publication year: 2023

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References

Simon, E, Long, B, Koyfman, A. Emergency medicine management of sickle cell disease complications: an evidence-based update. J Emerg Med 2016;51:370381.CrossRefGoogle ScholarPubMed
Ware, RE, de Montalembert, M, Tshilolo, L, et al. Sickle cell disease. Lancet 2017;390:311323.CrossRefGoogle ScholarPubMed

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