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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
Red blood cell transfusions during sickle cell anemia vaso-occlusive crises: a report from the magnesium in crisis
Monica L Hulbert1, Julie A Panepinto2, J Paul Scott2
1Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri.
Insights
Red blood cell (RBC) transfusions are seven times more likely in children with acute chest syndrome (ACS) during a sickle cell vaso-occlusive pain crisis (VOC). Lower hemoglobin levels, especially below 6.3 g/dL, also increase transfusion risk in children without ACS.
Area of Science:
- Pediatric Hematology
- Sickle Cell Disease Management
- Transfusion Medicine
Background:
- Red blood cell (RBC) transfusion practices for pediatric sickle cell vaso-occlusive pain crisis (VOC) are not well-understood.
- Hypothesized associations include acute chest syndrome (ACS), lower hemoglobin (Hb) levels, and lack of hydroxyurea therapy.
Purpose of the Study:
- To investigate RBC transfusion practices in children hospitalized with sickle cell VOC.
- To identify factors associated with RBC transfusion during VOC hospitalizations.
Main Methods:
- Secondary analysis of children with HbSS or S-β0 thalassemia in the MAGiC randomized trial.
- Prospective collection of ACS development and transfusion data.
- Analysis of Hb values and hydroxyurea use, comparing relative risks (RRs) of transfusion.
Main Results:
- 40 of 204 children (19.6%) received transfusions.
- Children with ACS had a sevenfold increased risk of transfusion (RR 7.1).
- In children without ACS, transfusion was associated with lower Hb levels (RR 3.1 per 1 g/dL decrease), with Hb < 6.3 g/dL being significant.
Conclusions:
- ACS significantly increases the likelihood of RBC transfusion in pediatric VOC.
- Low hemoglobin levels, particularly below 6.3 g/dL, are strongly associated with transfusion in non-ACS VOC patients.
- Hydroxyurea use was not found to be associated with transfusion practices in this cohort.
Background:
Little is known about red blood cell (RBC) transfusion practices for children hospitalized for a sickle cell vaso-occlusive pain crisis (VOC). We hypothesized that transfusion would be associated with the development of acute chest syndrome (ACS), lower hemoglobin (Hb) concentration, and lack of hydroxyurea therapy.
Study Design And Methods:
This is a secondary analysis of all children admitted for a sickle cell pain crisis enrolled in the Magnesium in Crisis (MAGiC) randomized trial; all had HbSS or S-β0 thalassemia. ACS development and transfusion administration were prospectively collected during the parent trial. All Hb values during the hospitalization were recorded, as was parent report of child receiving hydroxyurea. Relative risks (RRs) of transfusion were compared between groups.
Results:
Of 204 enrolled children, 40 (19.6%) received a transfusion. Of the 30 children who developed ACS, 22 (73.3%) received transfusions compared to 18 of 174 (10.3%) without ACS: the RR of transfusion in children with ACS was 7.1 (95% confidence interval [CI], 4.4-11.5). Among those without ACS, the lowest Hb was most strongly associated with transfusions: RR was 3.1 (95% CI 2.0 - 4.7) for each 1 g/dL decrease in lowest Hb. In a binary recursive partitioning model for those without ACS, a lowest recorded Hb level of less than 6.3 g/dL was significantly associated with transfusion during admission (p < 0.01). Hydroxyurea use was not associated with transfusions in any analysis.
Conclusion:
ACS increased the RR of transfusion in children hospitalized for VOC sevenfold. In children without ACS, transfusion was associated with lowest Hb concentration, particularly Hb concentration of less than 6.3 g/dL.
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