sickle cell anemia

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Related to sickle cell disease: sickle cell trait, Sickle cell crisis

sickle cell anemia

A chronic, severe, and sometimes fatal anemia marked by crescent-shaped red blood cells and characterized by fever, leg ulcers, jaundice, and episodic pain in the joints. The disease occurs in people who are homozygous for a gene that produces an abnormal form of hemoglobin, and it is found chiefly in people of African descent and in some Mediterranean, Middle Eastern, Central and South American, and South Asian populations. Also called sickle cell disease.
American Heritage® Dictionary of the English Language, Fifth Edition. Copyright © 2016 by Houghton Mifflin Harcourt Publishing Company. Published by Houghton Mifflin Harcourt Publishing Company. All rights reserved.

sick′le cell` ane′mia

a chronic hereditary blood disease, primarily affecting indigenous Africans and their descendants, in which an accumulation of oxygen-deficient sickle cells results in anemia, blood clotting, and joint pain.
Also called sicklemia.
Random House Kernerman Webster's College Dictionary, © 2010 K Dictionaries Ltd. Copyright 2005, 1997, 1991 by Random House, Inc. All rights reserved.

sick·le cell anemia

A hereditary disease characterized by red blood cells that are sickle-shaped instead of round because of an abnormality in the 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 oxygen available to those tissues.
Did You Know? Genetic mutations can be good or bad, and sometimes they can even be both. The mutation that causes sickle cell anemia is one example. It is harmful if a person inherits two copies of the mutated gene (one from each parent), but there is actually some 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. If a person's chromosomes have two copies of the mutated gene, serious sickle cell anemia results, causing illness. With just one copy of the gene, though, only some mild sickling of the cells occurs. It so happens that this mild sickling is harmful to the parasite that causes 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 malarial infection and to live long enough to have children, who then inherit the gene in turn. And because inheriting two copies of the gene is much less likely than inheriting just one, the benefits of the gene outweigh its risks for most people in these regions.
The American Heritage® Student Science Dictionary, Second Edition. Copyright © 2014 by Houghton Mifflin Harcourt Publishing Company. Published by Houghton Mifflin Harcourt Publishing Company. All rights reserved.
References in periodicals archive ?
It said that the neonatal screening of sickle cell disease forms part of the INTERREG CARES project, which aims to facilitate stronger collaboration in the health sector between the Eastern Caribbean and the French Associate Members of the OECS, namely Martinique and Guadeloupe.
Sickle cell disease is a genetic disorder that makes the bone marrow produce a defective protein that causes sickle-shaped blood cells to become hard and sticky. 
Sickle cell disease is a genetic disorder caused by sickle haemoglobin.
IMR-687 is an investigational, orally administered, highly potent and selective phosphodiesterase 9 inhibitor currently being evaluated as a potential disease-modifying therapeutic for sickle cell disease.
Imara Presented Positive, Interim Phase 2a Data on IMR-687 for the Treatment of Sickle Cell Disease at the 24th Congress of the European Hematology Association
Karolinska Development AB (STO:KDEV), a Nordic life sciences investment company, announced on Monday that its portfolio company, Modus Therapeutics (Modus), has reported results from a phase 2 study of sevuparin in patients with sickle cell disease.
Dr Zainab said that the national registry will cut down on time required to find blood for patients suffering from sickle cell disease and thalassaemia and will improve the management of sickle cell disease in Oman.
A bone marrow transplant is the only cure for sickle cell disease, as per the doctor, and has a success rate of 95 percent.
According to the company, Endari (L-glutamine oral powder) is indicated to reduce the acute complications of sickle cell disease in adult and paediatric patients five years of age and older.
Sickle cell disease; An overview of the disease and its systemic effects.

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