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Is there a threshold level of fetal hemoglobin that ameliorates morbidity
in sickle cell anemia?
DR Powars, JN Weiss, LS Chan and WA Schroeder
When the clinical manifestations of 272 patients with sickle cell anemia
are compared with their level of fetal hemoglobin (HbF), the results
suggest that there may be a threshold above which HbF is effective in
ameliorating the morbidity of this disease. The age of entry of these SS
patients into the study ranged from birth to 56 yr; the average length of
follow-up was 11 yr for a total of 3,011 patient- years of clinic
observation. HbF was determined quantitatively by microchromatographic
procedures; the mean for HbF was 10% +/- 6% with a range from 2% to 32%.
For major organ failure, analyzed as termination events of morbidity, such
as stroke or aseptic necrosis, the threshold appears to be 10%, whereas for
recurrent clinical events, such as crisis or pulmonary disorders, it is
20%. No linear trend was found between HbF levels and morbidity. If a
threshold exists, it is important to recognize this fact when attempts are
made to raise the level of HbF in patients with sickle cell disease.
Volume 63,
Issue 4,
pp. 921-926,
04/01/1984
Copyright © 1984 by The American Society of Hematology
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