This article is more than 5 years old. Information may no longer be current.
Doppler screening, intensification therapy reduced risk for stroke associated with sickle cell anemia
Bernaudin F. Blood. 2010;doi:10.1182/blood-2010-06-293514.
Click Here to Manage Email Alerts
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.
 |
Follow HemOncToday.com on Twitter. |