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Blood, Vol. 93 No. 1 (January 1), 1999:
pp. 71-79
Cerebral Vasculopathy in Sickle Cell Anemia: Diagnostic
Contribution of Positron Emission Tomography
Darleen R. Powars,
Peter S. Conti,
Wing-Yen Wong,
Paula Groncy,
Carol Hyman,
Elaine Smith,
Nadia Ewing,
Robert N. Keenan,
Chi-Shing Zee,
Yvonne Harold,
Alan L. Hiti,
Evelyn L. Teng, and
Linda S. Chan
From the Departments of Pediatrics, Radiology, Pathology and
Laboratory Medicine, and Neurology, the Divisions of
Hematology/Oncology and Biostatistics, University of Southern
California School of Medicine, Los Angeles; the Department of
Pediatrics, the Division of Hematology/Oncology, Long Beach Memorial
Hospital, Long Beach; the Ahmanson Pediatric Center, the Division of
Hematology/Oncology, Cedars Sinai Medical Center, Los Angeles; the
Department of Pediatrics, Hematology Division, Kaiser Foundation Health
Plan, Inc, Los Angeles; the Department of Pediatrics, City of Hope
Medical Center, Duarte, CA.
Children with sickle cell anemia (SS) have an increased risk for
cerebral vasculopathy with stroke (CVA) and cognitive impairment. The
present study examines the extent to which adding positron emission
tomography (PET) to magnetic resonance imaging (MRI) can improve the
detection of cerebral vasculopathy. Whereas MRI has been the prime
modality for showing anatomical lesions, PET excels at assessing the
functional metabolic state through glucose utilization 2-deoxy-2
[18F] fluoro-D-glucose (FDG) and microvascular blood flow
([15O]H2O). Forty-nine SS children were
studied. Among them, 19 had clinically overt CVA, 20 had
life-threatening hypoxic episodes or soft neurologic signs, and 10 were
normal based on neurological history and examination. For the entire
sample of 49 subjects, 30 (61%) had abnormal MRI findings, 36 (73%)
had abnormal PET findings, and 44 (90%) showed abnormalities on either
the MRI or the PET or both. Of the 19 subjects with overt CVA, 17 had abnormal MRI (89%), 17 had abnormal PET (89%), and 19 (100%) had either abnormal MRI or PET or both. Among the 20 subjects with soft
neurologic signs, 10 (50%) had abnormal MRI, 13 (65%) had abnormal
PET, and 17 (85%) had abnormal MRI and/or PET. Six (60%) of
the 10 neurologically normal subjects had abnormal PET. Among the 30 subjects with no overt CVA, 25 (83%) demonstrated imaging abnormalities based on either MRI or PET or both, thus, silent ischemia. Lower than average full-scale intelligence quotient (FSIQ) was associated with either overt CVA or silent
ischemic lesions. Four subjects who received chronic red blood
cell transfusion showed improved metabolic and perfusion
status on repeat PET scans. In conclusion, (1) the addition of PET to
MRI identified a much greater proportion of SS children with
neuroimaging abnormalities, particularly in those who had no history of
overt neurologic events. (2) PET lesions are more extensive, often
bihemispheric, as compared with MRI abnormalities. (3) PET may be
useful in management as a tool to evaluate metabolic improvement after
therapeutic interventions, and (4) the correlation of PET abnormalities
to subsequent stroke or progressive neurologic dysfunction requires
further study.

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