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Emergency: requires immediate attention
Sickle cell acute pain crisis
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Emergency: requires immediate attention

Sickle cell acute pain crisis

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Contributors: Abhijeet Waghray MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Sickle cell anemia is an autosomal recessive genetic disease. The genetic mutation results in an abnormal β-globin chain of hemoglobin A, and is known as hemoglobin S. Homozygote mutations for hemoglobin S manifest sickle cell anemia, a chronic disabling disease. The disease typically manifests during infancy at 4-5 months of age.

Around 70 000 people in the United States are affected by this disease. The incidence is greater in people of African and Caribbean descent and those from Mediterranean countries such as Turkey, Greece, and Italy. The incidence is equal in both males and females.

Hemoglobin S has diminished solubility and forms polymers, especially when there is low oxygen tension. This gives rise to the characteristic sickle shape of the affected red blood cells. Sickled cells are more prone to hemolysis and adherence to vascular endothelium, which leads to vascular occlusion, coagulation activation, and subsequent ischemia and tissue necrosis.

Acute presentations include:
  • Vaso-occlusive crises: painful episodes of microvascular occlusion that involve joints, bones, and internal organs such as the spleen, lungs (acute chest syndrome), liver, kidneys, and central nervous system.
  • Hematologic crises: due to pooling of blood in the enlarged spleen.
  • Infectious crises: result from functional asplenia, predisposing to infections from encapsulated organisms.
Acute chest syndrome is a leading cause of mortality in patients with sickle cell and is discussed separately.

See Sickle cell anemia for discussion of sickle cell leg ulcers and other more chronic manifestations.

Codes

ICD10CM:
D57.419 – Sickle-cell thalassemia with crisis, unspecified

SNOMEDCT:
416826005 – Sickle cell-thalassemia disease with crisis

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Last Reviewed: 03/28/2018
Last Updated: 04/19/2018
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Emergency: requires immediate attention
Sickle cell acute pain crisis
Print 1 Images
Sickle cell acute pain crisis : Bone pain, Arthralgia
Copyright © 2019 VisualDx®. All rights reserved.