Treatment

Blood transfusion is the standard treatment for sickle cell aplastic crises. Because the prognosis is so good, this may be done as an outpatient in developing countries.

Marrow aplasia is often the result of parvovirus B19 infection. In the normal individual the 120 day half life of a red blood cell means that a cessation in production for a few days does not have a significant effect. In the sickle cell patient the shorter circulating life of a red blood cell, combined with the aplasia, may result in dangerous anaemia.

Patients will usually have only one aplastic crisis (except in patients with severe immune deficiency) since a single parvovirus infection will give life long immunity against the disease (1).

A child is highly contagious during the time of aplasia. After diagnosis of aplastic crisisis is made the child should be isolated from the following vulnerable groups (2):

• pregnant women – since infection during second trimester may result in complications like hydrops fetalis and still births (1)
• immunocompromised children and all children with hemolytic anemias (2)

Parvovirus vaccination may be important in prophylaxis in the near future.

Reference:

• (1) Okpala I. The management of crisis in sickle cell disease. Eur J Haematol. 1998;60(1):1-6
• (2) Wethers DL. Sickle cell disease in childhood: Part II. Diagnosis and treatment of major complications and recent advances in treatment. Am Fam Physician. 2000;62(6):1309-14.