Silva et al evaluated children with sickle cell anemia for findings associated with complications and more severe disease. They identified a number of factors that can help to identify a patient who may require more aggressive management. The authors are from multiple institutions in Portugal.

Patient selection: child with sickle cell disease with age < 18 years


Patient selection: child with sickle cell disease


Predictors of severe sickle cell disease:

(1) baseline leukocytosis (WBC count > 15,000 per microliter)

(2) dactylitis during infancy (first year of life)


Predictors of less severe sickle cell disease:

(1) presence of alpha thalassemia


Other factors previously identified as being associated with more severe disease:

(1) low level of fetal hemoglobin (percent > 5.4% associated with reduced risk)


Therapy with hydroxyurea was associated with a better quality of life and long-term prognosis, even in the absence of clinical symptoms.

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