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Hydroxyurea increases hemoglobin F levels and improves the effectiveness of
erythropoiesis in beta-thalassemia/hemoglobin E disease
S Fucharoen, N Siritanaratkul, P Winichagoon, J Chowthaworn, W Siriboon, W Muangsup, S Chaicharoen, N Poolsup, B Chindavijak, P Pootrakul, A Piankijagum, AN Schechter and GP Rodgers
Department of Medicine, Faculty of Medicine, Siriraj Hospital, Bangkok,
Thailand.
Hydroxyurea (HU) is one of several agents that have been shown to enhance
hemoglobin (Hb) F levels in patients with sickle cell disease and may be
useful as a therapy for beta-globinopathies. However, limited information
exists on the effects of HU in patients with thalassemia. Accordingly, we
examined the hematologic effects of orally administered HU in 13 patients
with beta-thalassemia/Hb E, including four patients who had been
splenectomized. These patients were treated with escalating doses (final
range, 10 to 20 mg/kg/d) for 5 months and were observed in the outpatient
hematology clinic every 2 to 4 weeks. Complete blood counts including
reticulocyte counts, amounts of Hb E and Hb F, G gamma:A gamma and
alpha:non-alpha globin biosynthetic ratios were evaluated before and during
treatment. Almost all patients responded with an average increase of 33% in
Hb F levels, from a mean (+/- SD) of 42% +/- 11% to 56% +/- 8% (P <
.0001), and a reciprocal decline in the percentage of Hb E from 59% +/- 9%
to 49% +/- 8% (P < .001). Reticulocytosis was decreased from a mean (+/-
SD) of 18.0% +/- 15.6% to 11.7% +/- 9.1% (P < .05); there was also a
slight (10%) but statistically significant increase in hemoglobin levels
and an improved balance in alpha:non-alpha globin chains ratios. The side
effects were minimal in most patients, although these patients tended to
tolerate a lower dose of HU before significant myelosuppression than has
been our previous experience in sickle cell disease. One splenectomized
patient died of sepsis during the trial. We conclude that increased Hb F
production in beta-thalassemia/Hb E patients, with an improvement in the
alpha:non-alpha globin ratios and, probably, the effectiveness of
erythropoiesis, can be achieved using HU. Longer trials of HU in this
population, including at other doses and in combination with other agents,
appear warranted.
Volume 87,
Issue 3,
pp. 887-892,
02/01/1996
Copyright © 1996 by The American Society of Hematology
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