What complications can sickle cell anemia cause?

General: Because sickle cell disease (SCD) may decrease oxygen levels in the blood, organs and tissues may be deprived from oxygen and serious damage can result.

Vaso-occlusive crisis: If the abnormally shaped red blood cells cause blockages (called occlusions) in the small blood vessels (capillaries), vaso-occlusive crisis can occur. This can restrict blood flow to an organ, resulting in lack of blood (ischemia), organ damage, and pain. Vaso-occlusive crisis is also known as pain crisis.

Vaso-occlusive crisis often restricts blood to the bones and bone ischemia can result in serious bone weakness.

High blood pressure around the lungs, or pulmonary hypertension, is a serious complication of SCD. Pulmonary hypertension affects about 1/3 of adults with SCD and can result in heart failure.

Blocking of the blood vessels may affect the spleen, which is an important organ of the immune system. Damage to the spleen may require removal of the organ or autosplenectomy.

Acute chest crisis: A life-threatening complication of SCD is acute chest crisis or acute chest syndrome. The hallmarks of acute chest crisis include chest pain, shortness of breath, fever, hypoxemia (low oxygen in the blood), and abnormal chest X-rays. A particularly severe complication of acute chest crisis is atelectasis or collapsing of the lung. Vaso-occlusive (pain) crisis or respiratory infection may lead to acute chest syndrome.

Aplastic crisis: In patients with SCD, the bone marrow may temporarily stop producing red blood cells. This aplastic crisis usually occurs as a result of human parvovirus B19 infection. This infection often clears on its own and red blood cell production resumes. However, sometimes the number of red blood cells (hematocrit) falls so low that a blood transfusion is required. There is a high risk of other (secondary) infections during aplastic crisis, so individuals with aplastic crisis should be isolated to avoid secondary infections and the spreading of human parvovirus B19 infection.

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