Journal
AMERICAN JOURNAL OF HEMATOLOGY
Volume 83, Issue 6, Pages 482-484Publisher
WILEY
DOI: 10.1002/ajh.21130
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Funding
- NIDDK NIH HHS [R01 DK61883] Funding Source: Medline
- NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES [R01DK061883] Funding Source: NIH RePORTER
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beta-Thalassemia intermediate patients show a remarkable clinical heterogeneity. We examined the phenotypic diversity of 950 beta-thalassemia/Hb E patients in an attempt to construct a system for classifying disease severity. A novel scoring system based on six independent parameters, hemoglobin level, age at disease presentation, age at receiving first blood transfusion, requirement for transfusion, spleen size, and growth and development, was able to separate patients into three distinctive severity categories: mild, moderate, and severe courses. This system, therefore, can increase the accuracy of studies of genotype-phenotype interactions and facilitate decisions for appropriate patient management.
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