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A simple index for initiating transfusion treatment in thalassaemia intermedia
S Sbyrakis1, M Karagiorga-Lagana, I Voskaki
1School of Health Sciences, Division of Medicine, University of Crete, Iraklion, Greece.
Insights
Medullary width (MW) measurement can objectively identify thalassemia intermedia patients needing blood transfusions. This simple test helps determine optimal transfusion timing, potentially reversing bone deformities.
Area of Science:
- Hematology
- Radiology
- Pediatrics
Background:
- Thalassemia intermedia poses challenges in determining the need for blood transfusions.
- Objective markers are needed to guide transfusion decisions and manage complications like bone deformities.
Purpose of the Study:
- To evaluate medullary width (MW) and bone mass as objective tests for identifying thalassemia intermedia patients requiring blood transfusions.
- To assess the reversibility of bone deformities with timely transfusions based on MW measurements.
Main Methods:
- Measured medullary width (MW) and bone mass in normal children and thalassemia intermedia patients.
- Assessed hemoglobin (Hb), serum ferritin, and cephalofacial deformities (CFD) in patients.
- Monitored MW and bone mass changes before and after initiating blood transfusions based on MW criteria.
Main Results:
- Patients with thalassemia intermedia showed significantly different MW and bone mass compared to controls.
- A MW threshold of >0.5 cm identified patients who benefited from regular blood transfusions.
- Transfusions initiated based on MW >0.5 cm led to radiological improvements in MW and bone mass, suggesting reversibility of deformities.
Conclusions:
- Medullary width measurement is a simple, objective test to discriminate thalassemia intermedia patients needing transfusions.
- Using MW >0.5 cm as a criterion for initiating transfusions can help manage bone deformities effectively.
- This method aids clinicians in deciding the optimal time for regular transfusions in thalassemia intermedia.
Abstract:
In order to develop an objective test for discriminating between patients with thalassaemia intermedia requiring blood transfusion, and those not likely to require transfusion the medullary width (MW) in the midpoint of the second left metacarpal and the bone mass were measured in 34 normal children and in 37 patients. In patients, blood haemoglobin (Hb) and serum ferritin concentration were measured and cephalofacial deformities (CFD) were scored. The mean values of MW were 0.37 +/- 0.06 and 0.44 +/- 0.1 cm (P less than 0.01) and the bone mass 0.091 +/- 0.012 and 0.078 +/- 0.02 (P less than 0.005) in normal children and patients, respectively. In 13 of these patients who had MW more than 2 SD above the mean of the controls, i.e. more than 0.5 cm, regular blood transfusions were instituted. Measurements of MW 12 and 1 month before and 12 months after the initiation of transfusions showed an increase from 0.50 to 0.60 and a decrease to 0.49, respectively. Bone mass measured at the same times decreased from 0.083 to 0.045 and increased to 0.071, respectively. These changes were visible radiologically. It is concluded, therefore, that the measurement of MW seems to be an objective, simple test for discriminating between patients requiring or not blood transfusions, and that bone deformities will be reversible if transfusions are instituted using as criterion the MW (greater than 0.5 cm) regardless of age or haemoglobin concentration. This test may help clinicians to decide about the optimal time for institution of regular transfusions in patients with thalassaemia intermedia.