NEW YORK, Jan 12 (Reuters Health) -- Several markers of sickle cell
disease in the first two years of life can be used to predict severe
complications later in life, allowing doctors and families to make decisions
about higher risk treatments, researchers report.
"Ideally, the risks of these treatments should be commensurate with the
risks of untreated sickle cell disease, and potentially curative treatments
should begin before organ damage occurs," according to Dr. Scott T. Miller from
the State University of New York-Downstate Medical Center in Brooklyn, and
colleagues. Their report is published in the January 13th issue of The New
England Journal of Medicine.
Three easily detectable features of sickle cell disease in children under
2 years were found to predict severe disease later in life, the authors explain.
Children with severe anemia, or low red blood counts, faced more than twice the
risk of developing severe disease compared to children with higher blood counts.
Higher white blood cell counts, too, predicted severe disease, according
to the results.
A nearly threefold increase in severe sickle cell disease later in life
was related to the early development of dactylitis, a condition of painful,
swollen fingers resulting from the poor blood circulation associated with sickle
cell disease, the investigators report.
The highest risk was associated with the combination of severe anemia and
dactylitis or with one of these features plus a high white blood cell count, the
researchers note. Only about 3% of children fell into this high-risk group for
severe disease.
Miller and colleagues conclude that the triad of severe anemia, elevated
white blood cell count, and dactylitis in children under 2 years of age with
sickle cell disease can be used to predict the development of severe disease
before the age of 10 years.
The authors caution that their results should be used as a guide in
predicting severe sickle cell disease and not as a tool for diagnosing severe
disease. Nevertheless, they write, their findings should enable families and
physicians to assess the risks and benefits of high-risk therapies in the
context of the child's likelihood of developing severe disease.
