CHILDREN WITH SICKLE CELL ANEMIA ON CHRONIC TRANSFUSION FOR ABNORMAL TRANSCRANIAL DOPPLER VELOCITIES. PATIENT/SIBLING COMPARISON OF COGNITIVE PERFORMANCES AND ASSOCIATION WITH NEUROIMAGING DATA
(Abstract release date: 05/19/16)
EHA Library. Bernaudin F. 06/11/16; 135220; S464
Dr. Francoise Bernaudin
Contributions
Contributions
Abstract
Abstract: S464
Type: Oral Presentation
Presentation during EHA21: On Saturday, June 11, 2016 from 12:00 - 12:15
Location: Hall C14
Background
Patients with sickle cell anemia (SCA) are known to have cognitive deficiency. However, only a comparison with non-SCA siblings allows true appreciation of the disease impact.
Aims
The aims of the present study were to compare the cognitive performances of SCA-children on long-term transfusion programs due to a history of abnormal transcranial Doppler (TCD) velocities with those of their siblings, and to assess the relation to neuroimaging data.
Methods
Drepagreffe is a French national multicenter prospective trial (AP-HP, NCT 01340404) involving SCA-children, younger than 15 years of age, placed on chronic transfusion for abnormal-TCD (TAMMX ≥ 200 cm/sec), and comparing the outcome of cerebral vasculopathy following transfusions or hematopoietic stem cell transplant. We present here, the results of cognitive testings done at enrollment in SCA-children and non-SCA siblings, and patient neuroimaging data.The MRI scoring was: 3 = territorial, 2 = borderzone (cortical and subcortical), 1 = white matter or basal ganglia infarcts, 0 to 3 = atrophia, and MRA scoring was: 1 = mild stenosis (25-49%), 2 = moderate stenosis (50-74%), 3 = severe stenosis (75-99%), 4 = occlusion for each artery and 0 to 2 for Moya presence. Cognitive testing, performed in patients and in siblings when possible, included WPPSI-3 (3-6 yr), WISC-4 (7-16 yr) or WAIS-3 (>16 yr) scales, depending on the subject age.
Results
Sixty-seven SCA-children (36F/31M) were included. Seven had a history of overt stroke. At abnormal-TCD detection and chronic transfusion initiation, the mean age (SD) was 5.5yr (2.5) and velocities≥200/cm/sec were found in right/left middle (n=29/22), anterior (n=7/5), internal carotid (n=10/7) and extracranial internal carotid (n=7/7) arteries, as abnormal velocities were observed in more than one artery in several patients. At enrollment, following a mean duration of chronic transfusion of 2.8 years, the mean age was 8.1yr (3.1), and mean(SD) maximum velocities had significantly decreased from 219 (26) to 169 (46) cm/s (p<0.001). MRI/MRA data were available in 66/67 patients. Ischemic lesions were present in 25 patients and 18 of them had silent lesions. Stenoses were present in 35/66 patients in right/left middle (n=7/10), anterior (n=11/16), internal carotid (n=7/12) and extracranial internal carotid (n=11/12) arteries, as stenoses were observed in more than one artery in several patients. Cognitive testing was obtained in 64/67 patients (parental refusal for 3 patients), and in 56 siblings (8 were too young for testing). Paired analysis with siblings showed significant differences in Verbal Comprehension Index (VCI) with a mean difference of 7.6±14.5 (p<0.001), Processing Speed Index (PSI) 6.3±20.5 (p=0.04), and Full Scale IQ (FSIQ) 7.3±15.0 (p=0.001). Patient cognitive performance indexes were correlated negatively and significantly with the MRI and MRA scores . After exclusion of the 7 patients with stroke history, significant differences were still observed in VCI (p=0.013) and FSIQ (p=0.019), but were not correlated with the presence of silent cerebral infarcts.
Conclusion
SCA-children on transfusions for history of abnormal cerebral velocities had significantly lower cognitive performances than their siblings, even in the absence of stroke history. In the no-stroke patients, the differences observed with their siblings were not correlated with the presence of silent cerebral infarcts. These data suggest that stroke and silent infarcts are not the only underlying cause of cognitive deficiency in SCA children.
Session topic: Red blood cells and iron - Clinical
Keyword(s): Sickle cell anemia, Transcranial doppler
Type: Oral Presentation
Presentation during EHA21: On Saturday, June 11, 2016 from 12:00 - 12:15
Location: Hall C14
Background
Patients with sickle cell anemia (SCA) are known to have cognitive deficiency. However, only a comparison with non-SCA siblings allows true appreciation of the disease impact.
Aims
The aims of the present study were to compare the cognitive performances of SCA-children on long-term transfusion programs due to a history of abnormal transcranial Doppler (TCD) velocities with those of their siblings, and to assess the relation to neuroimaging data.
Methods
Drepagreffe is a French national multicenter prospective trial (AP-HP, NCT 01340404) involving SCA-children, younger than 15 years of age, placed on chronic transfusion for abnormal-TCD (TAMMX ≥ 200 cm/sec), and comparing the outcome of cerebral vasculopathy following transfusions or hematopoietic stem cell transplant. We present here, the results of cognitive testings done at enrollment in SCA-children and non-SCA siblings, and patient neuroimaging data.The MRI scoring was: 3 = territorial, 2 = borderzone (cortical and subcortical), 1 = white matter or basal ganglia infarcts, 0 to 3 = atrophia, and MRA scoring was: 1 = mild stenosis (25-49%), 2 = moderate stenosis (50-74%), 3 = severe stenosis (75-99%), 4 = occlusion for each artery and 0 to 2 for Moya presence. Cognitive testing, performed in patients and in siblings when possible, included WPPSI-3 (3-6 yr), WISC-4 (7-16 yr) or WAIS-3 (>16 yr) scales, depending on the subject age.
Results
Sixty-seven SCA-children (36F/31M) were included. Seven had a history of overt stroke. At abnormal-TCD detection and chronic transfusion initiation, the mean age (SD) was 5.5yr (2.5) and velocities≥200/cm/sec were found in right/left middle (n=29/22), anterior (n=7/5), internal carotid (n=10/7) and extracranial internal carotid (n=7/7) arteries, as abnormal velocities were observed in more than one artery in several patients. At enrollment, following a mean duration of chronic transfusion of 2.8 years, the mean age was 8.1yr (3.1), and mean(SD) maximum velocities had significantly decreased from 219 (26) to 169 (46) cm/s (p<0.001). MRI/MRA data were available in 66/67 patients. Ischemic lesions were present in 25 patients and 18 of them had silent lesions. Stenoses were present in 35/66 patients in right/left middle (n=7/10), anterior (n=11/16), internal carotid (n=7/12) and extracranial internal carotid (n=11/12) arteries, as stenoses were observed in more than one artery in several patients. Cognitive testing was obtained in 64/67 patients (parental refusal for 3 patients), and in 56 siblings (8 were too young for testing). Paired analysis with siblings showed significant differences in Verbal Comprehension Index (VCI) with a mean difference of 7.6±14.5 (p<0.001), Processing Speed Index (PSI) 6.3±20.5 (p=0.04), and Full Scale IQ (FSIQ) 7.3±15.0 (p=0.001). Patient cognitive performance indexes were correlated negatively and significantly with the MRI and MRA scores . After exclusion of the 7 patients with stroke history, significant differences were still observed in VCI (p=0.013) and FSIQ (p=0.019), but were not correlated with the presence of silent cerebral infarcts.
Conclusion
SCA-children on transfusions for history of abnormal cerebral velocities had significantly lower cognitive performances than their siblings, even in the absence of stroke history. In the no-stroke patients, the differences observed with their siblings were not correlated with the presence of silent cerebral infarcts. These data suggest that stroke and silent infarcts are not the only underlying cause of cognitive deficiency in SCA children.
Session topic: Red blood cells and iron - Clinical
Keyword(s): Sickle cell anemia, Transcranial doppler
Abstract: S464
Type: Oral Presentation
Presentation during EHA21: On Saturday, June 11, 2016 from 12:00 - 12:15
Location: Hall C14
Background
Patients with sickle cell anemia (SCA) are known to have cognitive deficiency. However, only a comparison with non-SCA siblings allows true appreciation of the disease impact.
Aims
The aims of the present study were to compare the cognitive performances of SCA-children on long-term transfusion programs due to a history of abnormal transcranial Doppler (TCD) velocities with those of their siblings, and to assess the relation to neuroimaging data.
Methods
Drepagreffe is a French national multicenter prospective trial (AP-HP, NCT 01340404) involving SCA-children, younger than 15 years of age, placed on chronic transfusion for abnormal-TCD (TAMMX ≥ 200 cm/sec), and comparing the outcome of cerebral vasculopathy following transfusions or hematopoietic stem cell transplant. We present here, the results of cognitive testings done at enrollment in SCA-children and non-SCA siblings, and patient neuroimaging data.The MRI scoring was: 3 = territorial, 2 = borderzone (cortical and subcortical), 1 = white matter or basal ganglia infarcts, 0 to 3 = atrophia, and MRA scoring was: 1 = mild stenosis (25-49%), 2 = moderate stenosis (50-74%), 3 = severe stenosis (75-99%), 4 = occlusion for each artery and 0 to 2 for Moya presence. Cognitive testing, performed in patients and in siblings when possible, included WPPSI-3 (3-6 yr), WISC-4 (7-16 yr) or WAIS-3 (>16 yr) scales, depending on the subject age.
Results
Sixty-seven SCA-children (36F/31M) were included. Seven had a history of overt stroke. At abnormal-TCD detection and chronic transfusion initiation, the mean age (SD) was 5.5yr (2.5) and velocities≥200/cm/sec were found in right/left middle (n=29/22), anterior (n=7/5), internal carotid (n=10/7) and extracranial internal carotid (n=7/7) arteries, as abnormal velocities were observed in more than one artery in several patients. At enrollment, following a mean duration of chronic transfusion of 2.8 years, the mean age was 8.1yr (3.1), and mean(SD) maximum velocities had significantly decreased from 219 (26) to 169 (46) cm/s (p<0.001). MRI/MRA data were available in 66/67 patients. Ischemic lesions were present in 25 patients and 18 of them had silent lesions. Stenoses were present in 35/66 patients in right/left middle (n=7/10), anterior (n=11/16), internal carotid (n=7/12) and extracranial internal carotid (n=11/12) arteries, as stenoses were observed in more than one artery in several patients. Cognitive testing was obtained in 64/67 patients (parental refusal for 3 patients), and in 56 siblings (8 were too young for testing). Paired analysis with siblings showed significant differences in Verbal Comprehension Index (VCI) with a mean difference of 7.6±14.5 (p<0.001), Processing Speed Index (PSI) 6.3±20.5 (p=0.04), and Full Scale IQ (FSIQ) 7.3±15.0 (p=0.001). Patient cognitive performance indexes were correlated negatively and significantly with the MRI and MRA scores . After exclusion of the 7 patients with stroke history, significant differences were still observed in VCI (p=0.013) and FSIQ (p=0.019), but were not correlated with the presence of silent cerebral infarcts.
Conclusion
SCA-children on transfusions for history of abnormal cerebral velocities had significantly lower cognitive performances than their siblings, even in the absence of stroke history. In the no-stroke patients, the differences observed with their siblings were not correlated with the presence of silent cerebral infarcts. These data suggest that stroke and silent infarcts are not the only underlying cause of cognitive deficiency in SCA children.
Session topic: Red blood cells and iron - Clinical
Keyword(s): Sickle cell anemia, Transcranial doppler
Type: Oral Presentation
Presentation during EHA21: On Saturday, June 11, 2016 from 12:00 - 12:15
Location: Hall C14
Background
Patients with sickle cell anemia (SCA) are known to have cognitive deficiency. However, only a comparison with non-SCA siblings allows true appreciation of the disease impact.
Aims
The aims of the present study were to compare the cognitive performances of SCA-children on long-term transfusion programs due to a history of abnormal transcranial Doppler (TCD) velocities with those of their siblings, and to assess the relation to neuroimaging data.
Methods
Drepagreffe is a French national multicenter prospective trial (AP-HP, NCT 01340404) involving SCA-children, younger than 15 years of age, placed on chronic transfusion for abnormal-TCD (TAMMX ≥ 200 cm/sec), and comparing the outcome of cerebral vasculopathy following transfusions or hematopoietic stem cell transplant. We present here, the results of cognitive testings done at enrollment in SCA-children and non-SCA siblings, and patient neuroimaging data.The MRI scoring was: 3 = territorial, 2 = borderzone (cortical and subcortical), 1 = white matter or basal ganglia infarcts, 0 to 3 = atrophia, and MRA scoring was: 1 = mild stenosis (25-49%), 2 = moderate stenosis (50-74%), 3 = severe stenosis (75-99%), 4 = occlusion for each artery and 0 to 2 for Moya presence. Cognitive testing, performed in patients and in siblings when possible, included WPPSI-3 (3-6 yr), WISC-4 (7-16 yr) or WAIS-3 (>16 yr) scales, depending on the subject age.
Results
Sixty-seven SCA-children (36F/31M) were included. Seven had a history of overt stroke. At abnormal-TCD detection and chronic transfusion initiation, the mean age (SD) was 5.5yr (2.5) and velocities≥200/cm/sec were found in right/left middle (n=29/22), anterior (n=7/5), internal carotid (n=10/7) and extracranial internal carotid (n=7/7) arteries, as abnormal velocities were observed in more than one artery in several patients. At enrollment, following a mean duration of chronic transfusion of 2.8 years, the mean age was 8.1yr (3.1), and mean(SD) maximum velocities had significantly decreased from 219 (26) to 169 (46) cm/s (p<0.001). MRI/MRA data were available in 66/67 patients. Ischemic lesions were present in 25 patients and 18 of them had silent lesions. Stenoses were present in 35/66 patients in right/left middle (n=7/10), anterior (n=11/16), internal carotid (n=7/12) and extracranial internal carotid (n=11/12) arteries, as stenoses were observed in more than one artery in several patients. Cognitive testing was obtained in 64/67 patients (parental refusal for 3 patients), and in 56 siblings (8 were too young for testing). Paired analysis with siblings showed significant differences in Verbal Comprehension Index (VCI) with a mean difference of 7.6±14.5 (p<0.001), Processing Speed Index (PSI) 6.3±20.5 (p=0.04), and Full Scale IQ (FSIQ) 7.3±15.0 (p=0.001). Patient cognitive performance indexes were correlated negatively and significantly with the MRI and MRA scores . After exclusion of the 7 patients with stroke history, significant differences were still observed in VCI (p=0.013) and FSIQ (p=0.019), but were not correlated with the presence of silent cerebral infarcts.
Conclusion
SCA-children on transfusions for history of abnormal cerebral velocities had significantly lower cognitive performances than their siblings, even in the absence of stroke history. In the no-stroke patients, the differences observed with their siblings were not correlated with the presence of silent cerebral infarcts. These data suggest that stroke and silent infarcts are not the only underlying cause of cognitive deficiency in SCA children.
Session topic: Red blood cells and iron - Clinical
Keyword(s): Sickle cell anemia, Transcranial doppler
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