Premium
A scoring system for the classification of β‐thalassemia/Hb E disease severity
Author(s) -
Sripichai Orapan,
Makarasara Wattanan,
Munkongdee Thongperm,
Kumkhaek Chutima,
Nuchprayoon Issarang,
Chuansumrit Ampaiwan,
Chuncharunee Suporn,
Chantrakoon Nawarat,
Boonmongkol Piathip,
Winichagoon Pranee,
Fucharoen Suthat
Publication year - 2008
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21130
Subject(s) - thalassemia , medicine , disease , beta thalassemia , phenotype , genotype , immunology , pediatrics , biology , genetics , gene
β‐Thalassemia intermediate patients show a remarkable clinical heterogeneity. We examined the phenotypic diversity of 950 β‐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. Am. J. Hematol. 2008. © 2008 Wiley‐Liss, Inc.