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A cautionary note regarding hydroxyurea in sickle cell disease
EP Vichinsky and BH Lubin
Department of Hematology/Oncology, Children's Hospital Oakland Research
Institute, CA 94609.
Hydroxyurea can increase fetal hemoglobin (HbF) and improve the clinical
course of sickle cell disease (SCD) patients. However, several issues of
hydroxyurea therapy remain unresolved, including differences in patients'
drug clearance, predictability of drug response, reversibility of sickle
cell disease-related organ damage by hydroxyurea, and the efficacy of
elevated HbF. We treated two patients with hydroxyurea for periods of 1 to
4 years, monitoring clinical course and laboratory parameters at regular
intervals. The first patient (patient A) had a history of chronic pain and
extensive hospitalizations. The second patient (patient B) had a history of
stroke and refused to continue with chronic transfusion therapy and
chelation. Both patients showed a fivefold to tenfold increase in HbF (5%
to 25%, 3% to 31%). However, patient A developed an acute chest syndrome,
despite an HbF level of 20%. After red blood cell transfusions for hypoxia,
the HbF level decreased to 5%. When hydroxyurea dosage was increased,
pancytopenia developed and was not resolved until 2 months after
hydroxyurea was discontinued; Patient B developed a cerebral hemorrhage on
hydroxyurea; he died shortly thereafter. His HbF level was 21% before
death. We noted an increase in HbF and a general improvement in the two
patients. However, both experienced major SCD-related complications despite
HbF levels over 20%. Our findings also suggest that the progressive
vascular changes associated with SCD are unlikely to be dramatically
affected by increased HbF levels. Because neither the efficacy nor the
toxicity of hydroxyurea have been thoroughly investigated, physicians
should be cautious in prescribing hydroxyurea for patients with SCD before
completion of the National Clinical Trial.
Volume 83,
Issue 4,
pp. 1124-1128,
02/15/1994
Copyright © 1994 by The American Society of Hematology
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