Plasma glial fibrillary acidic protein levels in a child with sickle cell disease and stroke

Abstract

A 12-year-old boy with HbSS sickle cell disease (SCD) was admitted with an acute febrile illness and developed overt stroke 3 days later. Plasma glial fibrillary acidic protein levels were elevated, as compared to pediatric controls, 32 h prior to the clinical diagnosis of stroke, peaked immediately prior to the exchange transfusion, and remained elevated 1 year later despite chronic transfusion therapy. Stroke in SCD can occur in the setting of acute illness, and a biomarker that could predict the onset and triage ill children to therapeutic intervention more quickly would be useful.

Fig. 1

Neuroimaging of acute stroke in a 12-year-old HbSS patient who had plasma GFAP followed serially. a Noncontrast head CT immediately after clinical diagnosis of stroke showing a wedge of hypoattenuation in the right frontal lobe (arrow). b FLAIR MRI image 29 h after clinical diagnosis of stroke showing an evolving right anterior cerebral artery infarct (arrow).

Fig. 2

a Serial plasma GFAP levels in a 12-year-old patient with HbSS and acute stroke. A red cell exchange transfusion was performed 5 h after clinical diagnosis and again 47 h later for new neurologic deficits. The dotted line marks the 95th percentile value among 60 healthy pediatric controls. Arrowheads mark the presentation of relevant signs and symptoms. b Fifty-four weeks of follow-up GFAP measurements.