sickle cell anemia

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.

sickle cell anemia

/ 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.

sickle cell anemia

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 .

Notes

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

Word History and Origins

Origin of sickle cell anemia¹

First recorded in 1925–30

A 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.

Example Sentences

In the 1960s in North Carolina, Adams and her family would take her sister Linda, who had sickle cell anemia, to the emergency room because they had no doctor and could not afford health insurance.

From Salon

An American teenager received a gene-editing treatment for his sickle cell anemia.

From Salon

Food and Drug Administration approved the first gene therapy treatment for sickle cell anemia, which works by interfering with an enhancer that results in activation of a fetal globin gene, reducing the production of sickled blood cells.

From Science Daily

One of her children is suffering from sickle cell anemia that often leaves her bed-ridden and in need of costly treatment and her youngest needs frequent medical attention after being scalded by boiling water around the torso.

From Seattle Times

"Our investigation provides powerful justifications for hydroxyurea's use in children with sickle cell anemia in Africa," said Dr. Chandy John, the Ryan White Professor of Pediatrics at IU School of Medicine and co-lead investigator of the latest study.

From Science Daily