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Doppler screening, intensification therapy reduced risk for stroke associated with sickle cell anemia
Bernaudin F. Blood. 2010;doi:10.1182/blood-2010-06-293514.
The combination of early screening with transcranial Doppler and intensification therapy reduced the cumulative risk for stroke by the age of 18 years from 11% to 1.9% in children with sickle cell anemia, French researchers have determined.
Researchers used transcranial Doppler (TCD) on 217 consecutive newborns diagnosed with homozygous sickle cell anemia, sickle cell/beta-zero thalassemia, sickle cell/D-Punjab and sickle cell/O-Arab at the Créteil Pediatric Sickle Cell Referral Center at the Créteil-Intercommunal-Hospital in Paris. Patients in the study were born from May 1988 to April 2007.
In 1,609 patient-years, the survival probability at 18 years was 97.5%. Overall incidence of stroke was 0.19 per 100 patient-years (95% CI, 0.04-0.5) and the cumulative risk for stroke by age 18 years was 1.9% (95% CI, 0.6-5.9).
There were 22 cases of stenosis, diagnosed at a median age of 4.8 years. Stenosis was bilateral in nine patients and unilateral in 13 patients. Cumulative risk for stenosis by age 14 years was 22.6% (95% CI, 15.0-33.2).
There were 109 patients who required intensification therapy. Eighty-six were placed on chronic transfusion for splenic sequestrations, frequent vaso-occlusive crisis and/or acute chest syndromes and abnormal TCD. There were no incidents of first stroke or relapse of high velocities observed during the 162 patient-years on chronic transfusion. However, in patients with a history of abnormal TCD and no TCD-normalization on chronic transfusion, researchers said four patients developed stenosis and one had a silent stroke.
Forty-five patients had abnormal TCD, so that in 149 patient-years post-abnormal TCD, there was a potential for 15 strokes. Only one patient had a stroke, which, researchers said, meant that 14 strokes were avoided by using intensive therapy.
Twenty-six patients underwent myeloablative geno-identical stem cell transplantation. Eighteen patients received bone marrow and eight received cord blood as a stem cell source. Indications were cerebral vasculopathy in 14 patients and frequent vaso-occlusive crisis and/or acute chest syndromes in 12 patients.
Engraftment was successful in 25 patients. None of the transplanted patients experienced a stroke, developed increased velocities or new ischemic lesions during the 98 patient-years follow-up.
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