Clinical characteristics of children with SCD with second overt strokes or progressive silent cerebral infarcts while receiving regular blood transfusion therapy for secondary prevention of strokes
| Patient no. . | Age at first stroke, y . | Type of progression . | Interval between first stroke and second event, y . | HbS at time of second overt stroke, % . | Mean HbS up to second overt stroke, % . | Total no. of overt strokes . | Total no. of MRIs with new or enlarging infarcts . | Clinical comments . | TIAs . | Vasculopathy on initial MRA . | Progression of vasculopathy on follow-up MRA . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 5.15 | TIA, then silent | 0.65 | NA | NA | 1 | 2 | MRI after TIA had new infarct—overt stroke? | One TIA reported | Occlusion L MCA/ACA, R ICA, MM | No |
| 2 | 6.31 | Silent | 3.65 | NA | NA | 1 | 1 | None reported | Severe R ICA, L ICA/MCA/ACA | Yes, + MM | |
| 3 | 3.73 | TIAs, then silent | 0.32 | NA | NA | 1 | 1 | MRI after first TIA had new infarct—overt stroke? | TIAs continued after RVS | Moderate R ICA/MCA/ACA, L MCA/ACA/PCA, MM | Yes, severe R MCA/ACA, L MCA/ACA |
| 4 | 3.77 | Overt | 0.75 | 48.1 | * | 4 | 3 | Missed appointment for transfusion week before second stroke | None reported | Severe R ICA/MCA/ACA/PCA, L MCA/ACA/PCA, MM | Yes, occlusion R ICA/ACA, severe L ICA |
| 5 | 4.38 | Overt | 1.98 | 10.2 | 22.9 | 3 | 2 | Yes | Occlusion R ICA, severe R MCA/ACA, L ICA/ACA/PCA, + MM | No change | |
| 6 | 9.16 | Silent | 6.32 | NA | NA | 1 | 1 | None reported | Occlusion R ICA/ACA, severe R MCA, severe L ICA/MCA/ACA, + MM | Yes, occlusion L ICA | |
| 7 | 2.40 | Overt | 0.57 | 16.6 | 17.3 | 2 | † | PFO closed by cardiac catheterization after second stroke | Several times per week | Normal | Yes, bilateral moyamoya |
| 8 | 3.68 | Overt | 0.70 | 28.2 | 30.6 | 2 | 1 | None reported | Moderate R ACA, mild R ICA, MCA | Yes, severe R ICA, MCA, ACA | |
| 9 | 5.80 | Silent, then overt | 3.53 (silent) 5.16 (overt) | 11.0 | 32.4 | 2 | 2 | Overt stroke after RVS that was done because of silent stroke | None reported | Severe R ICA, MCA, L MCA, + MM | Yes, occlusion R ICA, MCA |
| 10 | 8.71 | Overt | 2.89 | 38.0 | 43 | 3 | 1‡ | Had RVS after third stroke | None reported | Mild R ICA, MCA, severe L ICA, MCA, ACA, PCA | Yes, occlusion L ICA, MCA, ACA, + MM |
| 11 | 4.56 | Overt | 0.50 | * | * | 2 | † | None reported | † | † | |
| 12 | 4.89 | Overt | 1.50 | 21.2 | 30.4 | 2 | 1 | Every few months | Severe L ICA, MCA, ACA | Yes, moderate R ACA, mild R ICA, MCA, PCA | |
| 13 | 4.21 | Silent | 0.99 | NA | NA | 1 | 2 | None reported | Normal | Yes, severe R ICA, moderate R MCA, ACA, L ICA | |
| 14 | 3.41 | Silent, then TIAs | 3.41 | NA | NA | 1 | 1 | Silent infarct associated with cerebral AVM | Frequent after AVM resection | Severe R ICA, MCA, ACA, L ICA, MCA, ACA | No change |
| 15 | 3.47 | Silent | 3.96 | NA | NA | 1 | 1 | None reported | Severe R ICA, MCA, ACA, moderate L ICA | No change | |
| 16 | 3.68 | Silent | 7.76 | NA | NA | 1 | 1 | None reported | Normal | Severe R MCA, L MCA | |
| 17 | 5.07 | Silent | 3.89 | NA | NA | 1 | 1 | None reported | Normal | Normal | |
| 18 | 12.22 | Silent | 2.83 | NA | NA | 1 | 1 | None reported | Severe R ACA, L MCA, ACA | No change |
| Patient no. . | Age at first stroke, y . | Type of progression . | Interval between first stroke and second event, y . | HbS at time of second overt stroke, % . | Mean HbS up to second overt stroke, % . | Total no. of overt strokes . | Total no. of MRIs with new or enlarging infarcts . | Clinical comments . | TIAs . | Vasculopathy on initial MRA . | Progression of vasculopathy on follow-up MRA . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 5.15 | TIA, then silent | 0.65 | NA | NA | 1 | 2 | MRI after TIA had new infarct—overt stroke? | One TIA reported | Occlusion L MCA/ACA, R ICA, MM | No |
| 2 | 6.31 | Silent | 3.65 | NA | NA | 1 | 1 | None reported | Severe R ICA, L ICA/MCA/ACA | Yes, + MM | |
| 3 | 3.73 | TIAs, then silent | 0.32 | NA | NA | 1 | 1 | MRI after first TIA had new infarct—overt stroke? | TIAs continued after RVS | Moderate R ICA/MCA/ACA, L MCA/ACA/PCA, MM | Yes, severe R MCA/ACA, L MCA/ACA |
| 4 | 3.77 | Overt | 0.75 | 48.1 | * | 4 | 3 | Missed appointment for transfusion week before second stroke | None reported | Severe R ICA/MCA/ACA/PCA, L MCA/ACA/PCA, MM | Yes, occlusion R ICA/ACA, severe L ICA |
| 5 | 4.38 | Overt | 1.98 | 10.2 | 22.9 | 3 | 2 | Yes | Occlusion R ICA, severe R MCA/ACA, L ICA/ACA/PCA, + MM | No change | |
| 6 | 9.16 | Silent | 6.32 | NA | NA | 1 | 1 | None reported | Occlusion R ICA/ACA, severe R MCA, severe L ICA/MCA/ACA, + MM | Yes, occlusion L ICA | |
| 7 | 2.40 | Overt | 0.57 | 16.6 | 17.3 | 2 | † | PFO closed by cardiac catheterization after second stroke | Several times per week | Normal | Yes, bilateral moyamoya |
| 8 | 3.68 | Overt | 0.70 | 28.2 | 30.6 | 2 | 1 | None reported | Moderate R ACA, mild R ICA, MCA | Yes, severe R ICA, MCA, ACA | |
| 9 | 5.80 | Silent, then overt | 3.53 (silent) 5.16 (overt) | 11.0 | 32.4 | 2 | 2 | Overt stroke after RVS that was done because of silent stroke | None reported | Severe R ICA, MCA, L MCA, + MM | Yes, occlusion R ICA, MCA |
| 10 | 8.71 | Overt | 2.89 | 38.0 | 43 | 3 | 1‡ | Had RVS after third stroke | None reported | Mild R ICA, MCA, severe L ICA, MCA, ACA, PCA | Yes, occlusion L ICA, MCA, ACA, + MM |
| 11 | 4.56 | Overt | 0.50 | * | * | 2 | † | None reported | † | † | |
| 12 | 4.89 | Overt | 1.50 | 21.2 | 30.4 | 2 | 1 | Every few months | Severe L ICA, MCA, ACA | Yes, moderate R ACA, mild R ICA, MCA, PCA | |
| 13 | 4.21 | Silent | 0.99 | NA | NA | 1 | 2 | None reported | Normal | Yes, severe R ICA, moderate R MCA, ACA, L ICA | |
| 14 | 3.41 | Silent, then TIAs | 3.41 | NA | NA | 1 | 1 | Silent infarct associated with cerebral AVM | Frequent after AVM resection | Severe R ICA, MCA, ACA, L ICA, MCA, ACA | No change |
| 15 | 3.47 | Silent | 3.96 | NA | NA | 1 | 1 | None reported | Severe R ICA, MCA, ACA, moderate L ICA | No change | |
| 16 | 3.68 | Silent | 7.76 | NA | NA | 1 | 1 | None reported | Normal | Severe R MCA, L MCA | |
| 17 | 5.07 | Silent | 3.89 | NA | NA | 1 | 1 | None reported | Normal | Normal | |
| 18 | 12.22 | Silent | 2.83 | NA | NA | 1 | 1 | None reported | Severe R ACA, L MCA, ACA | No change |
NA indicates HbS not applicable as progressive infarction was clinically silent; RVS, revascularization surgery; L, left; R, right; MCA, middle cerebral artery; ACA, anterior cerebral artery; ICA, internal carotid artery; PCA, posterior cerebral artery; PFO, patent foramen ovale; and AVM, arteriovenous malformation.
HbS data not available.
Serial MRI not available.
Third stroke was clinical with no change in MRI on that date.