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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
Magnitude of elevated iron stores and risk associated in steady state sickle cell anemia Congolese children: a cross
Jean-Robert Makulo1, Karen Efombola Itokua2, Rosette Kevani Lepira2
11Division of Nephrology, Nephrology Unit, Department of Internal Medicine, University Hospital of Kinshasa, Faculty of Medicine, University of Kinshasa, Kinshasa XI, PO.BOX 123, Democratic Republic of Congo.
Insights
In Sickle Cell Anemia (SCA) children, elevated iron stores are common, affecting nearly one in five patients. Frequent blood transfusions are the primary risk factor, highlighting the need for better iron monitoring and management in these patients.
Area of Science:
- Hematology
- Pediatrics
- Public Health
Background:
- Serum ferritin assays are crucial for monitoring iron stores in Sickle Cell Anemia (SCA) patients undergoing regular transfusions.
- Elevated iron stores, indicated by serum ferritin ≥500 ng/ml, are typically associated with iron chelation therapy after numerous transfusions.
- The prevalence and risk factors for elevated iron stores in Congolese children with SCA were previously underexplored.
Purpose of the Study:
- To determine the prevalence of elevated iron stores in Congolese children with steady-state SCA.
- To identify risk factors associated with elevated iron stores in this pediatric SCA population.
- To assess the magnitude of hyperferritinemia requiring iron chelation in SCA children.
Main Methods:
- Serum ferritin levels were measured in 70 steady-state SCA children across two Kinshasa hospitals.
- Elevated iron stores were defined as serum ferritin levels ≥500 ng/ml.
- Univariate analysis was employed to identify associated risk factors for hyperferritinemia.
Main Results:
- 21.4% of SCA children exhibited elevated iron stores (serum ferritin ≥500 ng/ml).
- Patients with elevated iron stores had a higher frequency of polytransfusions (more than 3 in the last year) compared to those without (73% vs. 44%).
- Receiving more than 3 transfusions annually was the main risk factor for elevated iron stores, with an odds ratio of 6.17.
Conclusions:
- Hyperferritinemia necessitating iron chelation in SCA children is strongly linked to blood transfusions.
- The study highlights that nearly one in five SCA children in the cohort had elevated iron stores, indicating a significant and potentially underestimated problem.
- Findings underscore the importance of regular iron monitoring and timely intervention in transfused SCA patients to prevent iron overload.
Background:
The serum ferritin assay is recommended in Sickle Cell Anemia (SCA) patients receiving regular transfusions. According to several authors, elevated iron stores indicating iron chelation corresponds to hyperferritinemia ≥500 ng/ml, and becomes detectable after twenty blood transfusions. The objectives of the study were to determine the prevalence of elevated iron stores and identify associated risk factors in a case series of Steady state SCA Congolese children.
Material And Methods:
Serum ferritin was assayed in Steady state SCA children followed in 2 specialized hospitals in Kinshasa. Elevated iron stores was defined as serum ferritin level ≥ 500 ng/ml, and the associated risk factors were identified using univariate analysis.
Results:
Seventy patients (median age 9 years, 56% boys, 53% receiving hydroxyurea) were selected in the study. Serum ferritin levels ranged from 24 to 2584 ng / ml with 21.4% of children having elevated iron stores. Mean levels of LDH, indirect bilirubin, plasma free Hb and CRP were similar between the 2 groups whereas history of polytransfusions (> 3 during the last year) was more frequent among patients with elevated iron stores (73% vs. 44%, p = 0.078). Receiving > 3 transfusions in a year vs. 0 was the main risk factor associated with elevated iron stores [OR 6.17 (95% CI: 1.81-20.96)].
Conclusion:
In SCA children, hyperferritinemia requiring iron chelation is most strongly related to blood transfusion. This situation concerned almost one in five children in present study; this shows the magnitude of the problem which is underestimated.
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