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sickle cell disease

Introduction

sickle cell disease or sickle cell anemia, inherited disorder of the blood in which the oxygen-carrying hemoglobin pigment in erythrocytes (red blood cells) is abnormal. This "hemoglobin-S" crystallizes in small capillaries, where the concentration of oxygen in the blood is low (but sufficient for normal hemoglobin), causing the red blood cells to assume distorted, sicklelike shapes. Linus Pauling discovered the chemical abnormality of the hemoglobin molecule that causes the erythrocyte sickling in 1949.

The sickled red blood cells tend to clog small blood vessels, depriving the tissues they serve of blood and oxygen. Painful "crises" result, with symptoms depending on the site affected (e.g., joint and abdominal pain or kidney damage). Strokes or seizures can occur if the brain is affected. Lung infections resulting from the patient's disinclination to take painful deep breaths are a frequent complication. In addition, the sickled erythrocytes are fragile and subject to rupture and destruction, leading to hemolytic anemia (reduction of oxygen-carrying hemoglobin caused by premature destruction of red blood cells) and such symptoms as fatigue, jaundice, and headaches.

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The Columbia Electronic Encyclopedia, 6th ed. Copyright © 2012, Columbia University Press. All rights reserved.

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