Sickle cell anemia with systemic lupus erythematosus: response to hydroxyurea therapy.

PURPOSE: To report the efficacy of hydroxyurea (HU) in a patient with sickle cell anemia (SCA) associated with systemic lupus erythematosus (SLE).

PATIENTS AND

METHODS

An unusual association of SCA with SLE occurred in a 14-year-old black girl. Her medical history was significant for repeated vasoocclusive crises requiring repeated hospitalizations and transfusions, acute chest syndrome, hyperhemolytic crises, multiple transfusions resulting in iron overload, gall stones, avascular necrosis, and significant psychosocial problems. Her lupus activity was controlled with oral prednisone and hydroxychloroquine. Treatment with oral HU was instituted at an initial dose of 20 mg/kg with gradual increment to a maximum of 25 mg/kg daily.

RESULTS

A dramatic clinical improvement was noted with HU therapy with no episode of pain crisis or hospitalization for 5 months. There was an increase in fetal hemoglobin (HbF) to 20.9% and mean corpuscular volume to 114.5 fl, but her Hb level remained steady at 7.5 g/dl. No toxicity was noted with HU therapy. In addition, a significant change was also observed in her school performance, social activities, and general quality of life.

CONCLUSIONS

HU therapy may be beneficial and safe and should be considered for other patients who have SCA with SLE.