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reference materials

Sickle Cell Anemia

key information

source: Orphanet

year: 2007


Sickle cell anemias are chronic hemolytic diseases that may induce three types of acute accidents: severe anemia, severe bacterial infections, and ischemic vasoocclusive accidents (VOA) caused by sickle-shaped red blood cells obstructing small blood vessels and capillaries. Many diverse complications can occur.


The prevalence of sickle cell carriers in 25 European states is estimated at about 1/150. In central and western Africa (15-25%), in the French West Indies (10-15%) and in Mediterranean areas (1-15%) a high prevalence is observed in areas that are or have been affected by malaria, because the trait offers protection against pernicious malaria.

Clinical description:

The presence of fetal hemoglobin means that the disease doesn’t manifest until after 3 months. Clinical manifestations are extremely variable between individuals and at different times. In addition to anemia and bacterial infections, VOAs cause hyperalgic focal ischemia (and sometimes infarction) when they occur in the muscles or skeleton. Over the course of time, VOAs may compromise the integrity of tissues or organs.

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