sickle cell anemia

American  

noun

Pathology.

1. a chronic hereditary blood disease, occurring primarily among Africans or persons of African descent, in which abnormal hemoglobin causes red blood cells to become sickle-shaped and nonfunctional, characterized by enlarged spleen, chronic anemia, lethargy, weakness, joint pain, and blood clot formation.

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sickle cell anemia Scientific  

/ sĭk′əl /

1. A hereditary disease characterized by red blood cells that are sickle-shaped instead of round because of an abnormality in their hemoglobin, the protein that carries oxygen in the blood. Because of their shape, the cells can cause blockage of small blood vessels in the organs and bones, reducing the amount of available oxygen.

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sickle cell anemia Cultural  

1. A hereditary form of anemia in which the red blood cells become sickle-shaped (shaped like a crescent) and less able to carry oxygen.

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Closer Look

Sickle cell anemia is a genetic mutation that can be either detrimental or beneficial depending on the number of copies of the mutated gene a person inherits. While it is harmful if a person inherits two copies of the mutated gene (one from each parent), a person could actually benefit if only one copy of the gene is inherited. The defective gene causes red blood cells to be distorted into a sickle shape, which makes it hard for them to pass through the tiny blood vessels where they give oxygen to body tissues. Inheriting two copies of the mutated gene results in a lifelong disease that causes anemia, pain, and other complications. With just one copy of the gene, though, only mild sickling occurs, and the disease does not manifest itself. This mild sickling, however, is also harmful to the parasites that cause malaria and can protect a person from that disease. In a region like tropical Africa where malaria is common, people who have the mutation in one gene are more likely to ward off a malaria infection and to live long enough to have children, who then inherit the gene. And because a person is less likely to inherit two copies of the gene instead of just one, the benefits of the gene outweigh its risks for most people in these regions. About one in 500 African-American newborns and one out of every 1,000 to 1,400 Hispanic babies are diagnosed with sickle cell anemia each year in the United States. Almost ten percent of African Americans carry the sickle cell gene. There is no cure for the disease, but treatment can reduce pain and prolong life.

Discover More

Sickle cell anemia is a chronic disease and occurs most frequently in people of African descent.

Etymology

Origin of sickle cell anemia

First recorded in 1925–30

Compare meaning

How does sickle-cell-anemia compare to similar and commonly confused words? Explore the most common comparisons:

• sickle cell anemia vs. anemia

Example Sentences

"The first gene editing drug approved for a blood disease -- sickle cell anemia -- targets a cell-type specific enhancer."

From Science Daily

Cases include electrocution, drowning, overdose, scurvy, sickle cell anemia, a nail in the chest, a fastball in the eye and gallstones, with all the personal drama one expects from a hospital show.

From Los Angeles Times

The doctors will take on cases of electrocution, drowning, overdose, trauma, scurvy, sickle cell anemia, a nail in the chest, a fastball in the eye, gallstones, third-degree burns, chlamydia, a faulty pacemaker, rats in the clothes of an unhoused man, and on and on, each with a story and a backstory.

From Los Angeles Times

Last May, a 12-year-old with sickle cell anemia was the first person to receive a new gene therapy to treat the disease.

From Slate

Guests: Gina Kolata, medical reporter for the New York Times Deb and Keith Cromer, parents to Kendric Cromer, the first person in the world to go through a commercially approved gene therapy for sickle cell anemia.

From Slate