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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Discontinuing prophylactic transfusions increases the risk of silent brain infarction in children with sickle cell
Miguel R Abboud1, Eunsil Yim, Khaled M Musallam
1American University of Beirut Medical Center, Beirut, Lebanon. abboudm@aub.edu.lb
Insights
Discontinuing blood transfusions in children with sickle cell disease (SCD) and abnormal TCD scans increased the risk of silent brain infarcts. Transfusions are crucial for preventing these brain injuries in high-risk children.
Area of Science:
- Pediatric Neurology
- Hematology
- Radiology
Background:
- Sickle cell disease (SCD) poses a significant risk for cerebrovascular complications, including silent brain infarcts.
- Transcranial Doppler (TCD) ultrasonography is used to identify children with SCD at high risk for stroke.
- Prophylactic blood transfusions are a standard intervention for preventing stroke in these children.
Purpose of the Study:
- To investigate the impact of discontinuing prophylactic transfusions on the development and progression of silent brain infarcts detected by magnetic resonance imaging (MRI) in children with SCD.
- To analyze data from the STOP II trial to assess the relationship between transfusion status and MRI-detected brain lesions.
Main Methods:
- Analysis of data from the STOP II trial, including baseline and end-of-study MRI scans.
- Comparison of new brain MRI lesions and changes in lesion counts between children who continued transfusions and those who halted them.
- Assessment of baseline characteristics to ensure comparability between study groups.
Main Results:
- At study entry, 27% of patients had evidence of silent infarcts on MRI.
- New brain MRI lesions developed in 8.1% of patients in the continued-transfusion group versus 27.5% in the transfusion-halted group (P = .03).
- The number of lesions increased significantly in the transfusion-halted group (from 27 to 45) but remained stable in the continued-transfusion group (from 25 to 24).
Conclusions:
- Discontinuation of prophylactic transfusions in children with SCD and abnormal TCD velocities, even if they revert to low-risk, increases the risk of developing silent brain infarcts.
- These findings, along with data from the STOP trial, confirm that transfusions effectively prevent silent infarcts in SCD patients with abnormal TCD but normal MRA.
- Maintaining prophylactic transfusions is essential for preventing cerebrovascular complications in high-risk children with SCD.
Abstract:
In the STOP II trial, discontinuation of prophylactic transfusions in high risk children with sickle cell disease (SCD) resulted in a high rate of reversion to abnormal blood-flow velocities on transcranial Doppler (TCD) ultrasonography and strokes. We analyzed data from STOP II to determine the effect of discontinuing transfusions on the development or progression of silent brain infarcts on magnetic resonance imaging (MRI). At study entry, 21 of 79 (27%) patients had evidence of silent infarcts. There were no statistically significant differences in baseline characteristics between patients with normal brain MRI or silent infarcts at study entry. At study end, 3 of 37 (8.1%) patients in the continued-transfusion group developed new brain MRI lesions compared with 11 of 40 (27.5%) in the transfusion-halted group (P = .03). The total number of lesions remained essentially unchanged decreasing from 25 to 24 in the continued-transfusion group while increasing from 27 to 45 in transfusion-halted patients. Thus, discontinuation of transfusions in children with SCD and abnormal TCD who revert to low-risk increases the risk of silent brain infarction. Together with data from STOP, these findings demonstrate that transfusions prevent the development of silent infarcts in patients with SCD and abnormal TCD but normal MRA.
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