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Sickle Cell Disease: Clinical Features

Life Expectancy

Chronic hemolytic anemia, interrupted by acute and recurrent crises that vary greatly in frequency and severity, is common. In its most severe form, sickle cell disease (SCD) has a significant impact on morbidity and mortality.

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In its more severe form, sickle cell disease has a significant impact on morbidity and mortality

As recently as the 1970s, a patient with SCD was not expected to survive to adulthood. Improvements in patient care have had a significant influence on prognosis, with a substantial increase in life expectancy. Among individuals with the homozygous sickle hemoglobin mutation (HbSS), the median age of death is 42 years for men and 48 years for women.1

Vaso-Occlusive Crises

The chronic hemolysis of SCD-associated anemia is punctuated by acute exacerbations of the illness, termed sickling. The most common manifestations are the vaso-occlusive crises, characterized by acute painful episodes due to blockage of small vessels with sickled erythrocytes, and tissue infarction.2

Vaso-occlusion is also dependent on cell factors, such as the state of the vascular endothelium, vascular tone, and activation of platelets and white blood cells.

Repeated vaso-occlusive events ultimately result in end-organ damage and almost any organ can be affected (Figure).

Clinical Manifestations of SCD

Figure. Clinical manifestations of SCD.


Learn more about SCD in children >

References

  1. Platt OS, et al. N Engl J Med. 1994;330:1639-44.
  2. Schnog JB, et al. Neth J Med. 2004;62:364-74.

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