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Sickle Cell Anemia

Sickle cell anemia is an inherited disorder transmitted via a recessive gene. As a result of genetic dysfunction, red blood cells may change shape under certain circumstances. The odd shapes of blood cells may cause blockage of capillaries which deprives the downstream tissue of oxygen and causes ischemia and infarction.

Sickle cell anemia symptoms result from the shortened life span of the abnormal red cells, resulting in a chronic anemia. The appearance of being pale, weakness, and fatigue are common symptoms as well. Jaundice may result from the breaking down of red cells prior to processing in the liver. In the peripheral tissue, red cells may create thrombi formation and vascular occlusion.

Looking at the clinical symptoms of sickle cell anemia, when not acute, patients have baseline anemia that varies in severity. Often times white blood cell counts are elevated simply due to the fact that the marrow is hyperactivity as the body needs to replace the sickled red cells, which have a shorter life than normal red blood cells.

vasoocclusive crises - blockage of capillaries due to sickle cell anemia

Vasoocclusive crises are caused by sickled red blood cells which obstruct capillaries and thus block blood flow to an organ. The resulting damage may include; ischemia, pain, and organ damage.

Among the most common areas targeted by vasoocclusive crisis includes:

  • Spleen
  • Liver
  • Bone
  • Lungs

Posted by Staff at July 8, 2005 4:40 AM

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