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EVALUATION OF INTRACRANIAL CEREBRAL BLOOD FLOW VELOCITIES IN SPLENECTOMIZED AND NON-SPLENECTOMIZED ?-THALASSEMIA INTERMEDIA PATIENTS USING TRANSCRANIAL DOPPLER SONOGRAPHY
Author(s): ,
Aikaterini Kanavaki
Affiliations:
First Department of Pediatrics,University of Athens Medical School,Athens,Greece
,
Polyxeni Delaporta
Affiliations:
First Department of Pediatrics,University of Athens Medical School,Athens,Greece
,
Dimitra Kyriakopoulou
Affiliations:
First Department of Pediatrics,University of Athens Medical School,Athens,Greece
,
Ioannis Papasotiriou
Affiliations:
Department of Clinical Biochemistry, ‘Aghia Sophia’ Children’s Hospital,Athens,Greece
,
Konstantinos Spengos
Affiliations:
First Department of Neurology,University of Athens Medical School,Athens,Greece
Antonis Kattamis
Affiliations:
First Department of Pediatrics,University of Athens Medical School,Athens,Greece
(Abstract release date: 05/19/16) EHA Library. KATTAMIS A. 06/09/16; 134958; PB2058
Prof. Antonis KATTAMIS
Prof. Antonis KATTAMIS
Contributions
Abstract
Abstract: PB2058

Type: Publication Only

Background
Beta-thalassemia intermedia (βΤΙ) is a congenital hemolytic anemia with a widely variable clinical phenotype. A high incidence of clinically silent cerebral ischemic events (SCI) has been reported in splenectomized β-ΤI patients. These could be due to cerebral large-vessel disease. 

Aims
Based on the example of sickle cell disease, we applied transcranial Doppler sonography (TCD) to evaluate cerebral vessels velocity as a possible indicator of cerebral vasculopathy in these patients.

Methods
We included in our study 30 patients with βΤΙ, 17 patients with splenectomy (group A) and 13 non splenectomised ones (group B), aged between 13 and 63 years-old ( median: 34, mean: 40)Non imaging TCD was performed in all patients and the TAMV values in the anterior (ACA), middle (MCA), posterior (PCA) cerebral and the basilar  (BA) artery were measured. Nine of the patients have been also examined by brain MRI/MRA (7 patients from group A and 2 patients from group B).

Results
None of the patients had a TAMV value ≥ 200cm /sec. In group A the maximum TAMV of the  examined vessels were 104 cm/sec MCA R, 139 cm/sec MCA L, 89 cm /sec ACA R, 93 cm/ sec ACA L, 59 cm /sec PCA R, 64 cm / sec PCA L, 75 cm / sec BA. In the same group the mean and median values were 72.4/ 75 cm/sec MCA R, 72/72.5 cm/sec MCA L, 66.3/67 cm /sec ACA R, 59/57 cm / sec ACA L, 43.5/43 cm/ sec PCA R, 43.5/ 41 cm /sec PCA L, 55.5/ 55 cm /sec BA respectively.In group B the maximum TAMV were 88 cm/sec MCA R, 93 cm/sec MCA L, 98 cm /sec ACA R, 93 cm/ sec ACA L, 55 cm /sec PCA R, 59 cm / sec PCA L, 68 cm / sec BA.In the same group the mean and median values were 69.6/ 66 cm/sec MCA R, 76.5/78 cm/sec MCA L, 69.7/69 cm /sec ACA R, 56.7/57 cm / sec ACA L, 38.5/37 cm/ sec PCA R, 43.6/44 cm /sec PCA L, 47.1/ 46.5 cm /sec BA respectively.We found no statistically significant difference (p>0.05) in the TAMV values for all examined vessels between the two groups.No statistically significant difference was found between the two groups concerning age, gender, hemoglobin and hematocrit levels.There was a statistically significant difference in PLT blood count that was elevated in splenectomised β-ΤI patients (p<0.01) as it was expected.From the patients who were also examined by MRI only one had a SCI lesion on MRI, but without any abnormality on TOF-MR angiography. 

Conclusion
Our results do not show increased TAMV velocities and do not support the presence of large vessels vasculopathy in splenectomised β-ΤI patients. These findings agree with recent published data according to which SCIs in these patients might be due to microangiopathy or venous thromboembolism.

Session topic: E-poster

Keyword(s): Beta thalassemia, Splenectomy, Transcranial doppler
Abstract: PB2058

Type: Publication Only

Background
Beta-thalassemia intermedia (βΤΙ) is a congenital hemolytic anemia with a widely variable clinical phenotype. A high incidence of clinically silent cerebral ischemic events (SCI) has been reported in splenectomized β-ΤI patients. These could be due to cerebral large-vessel disease. 

Aims
Based on the example of sickle cell disease, we applied transcranial Doppler sonography (TCD) to evaluate cerebral vessels velocity as a possible indicator of cerebral vasculopathy in these patients.

Methods
We included in our study 30 patients with βΤΙ, 17 patients with splenectomy (group A) and 13 non splenectomised ones (group B), aged between 13 and 63 years-old ( median: 34, mean: 40)Non imaging TCD was performed in all patients and the TAMV values in the anterior (ACA), middle (MCA), posterior (PCA) cerebral and the basilar  (BA) artery were measured. Nine of the patients have been also examined by brain MRI/MRA (7 patients from group A and 2 patients from group B).

Results
None of the patients had a TAMV value ≥ 200cm /sec. In group A the maximum TAMV of the  examined vessels were 104 cm/sec MCA R, 139 cm/sec MCA L, 89 cm /sec ACA R, 93 cm/ sec ACA L, 59 cm /sec PCA R, 64 cm / sec PCA L, 75 cm / sec BA. In the same group the mean and median values were 72.4/ 75 cm/sec MCA R, 72/72.5 cm/sec MCA L, 66.3/67 cm /sec ACA R, 59/57 cm / sec ACA L, 43.5/43 cm/ sec PCA R, 43.5/ 41 cm /sec PCA L, 55.5/ 55 cm /sec BA respectively.In group B the maximum TAMV were 88 cm/sec MCA R, 93 cm/sec MCA L, 98 cm /sec ACA R, 93 cm/ sec ACA L, 55 cm /sec PCA R, 59 cm / sec PCA L, 68 cm / sec BA.In the same group the mean and median values were 69.6/ 66 cm/sec MCA R, 76.5/78 cm/sec MCA L, 69.7/69 cm /sec ACA R, 56.7/57 cm / sec ACA L, 38.5/37 cm/ sec PCA R, 43.6/44 cm /sec PCA L, 47.1/ 46.5 cm /sec BA respectively.We found no statistically significant difference (p>0.05) in the TAMV values for all examined vessels between the two groups.No statistically significant difference was found between the two groups concerning age, gender, hemoglobin and hematocrit levels.There was a statistically significant difference in PLT blood count that was elevated in splenectomised β-ΤI patients (p<0.01) as it was expected.From the patients who were also examined by MRI only one had a SCI lesion on MRI, but without any abnormality on TOF-MR angiography. 

Conclusion
Our results do not show increased TAMV velocities and do not support the presence of large vessels vasculopathy in splenectomised β-ΤI patients. These findings agree with recent published data according to which SCIs in these patients might be due to microangiopathy or venous thromboembolism.

Session topic: E-poster

Keyword(s): Beta thalassemia, Splenectomy, Transcranial doppler

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