Sickle cell acute pain crisis
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.
See Sickle cell anemia for discussion of sickle cell leg ulcers and other more chronic manifestations.
D57.419 – Sickle-cell thalassemia with crisis, unspecified
416826005 – Sickle cell-thalassemia disease with crisis
- Parvovirus B19 infection
- Bone infarcts
- Gout / septic arthritis
- Alpha and beta thalassemia
- Glucose-6-phosphate dehydrogenase deficiency (G6PD)
- Systemic lupus erythematosus
- Drug-related hemolytic anemia
- Disseminated intravascular coagulation
- Hemolytic uremic syndrome
- Thrombotic thrombocytopenic purpura
Last Updated: 04/19/2018