Phadke and Agarwal reported the phenotype score for thalassemia intermedia. The authors are from Sanjay Gandhi Post Graduate Institute of Medical Sciences in Lucknow, India.
Patient selection: thalassemia intermedia
Parameters:
(1) age at presentation
(2) mean hemoglobin in g/dL
(3) growth retardation (height less than 3rd percentile for age and gender)
(4) thalassemia facies
(5) age at first blood transfusion
(6) frequency of blood transfusion
Parameter
Finding
Points
age at presentation
< 6 years
2
6 to 15 years
1
> 15 years
0
hemoglobin
6 to 7.5 g/dL
2
7.5 to 9.5 g/dL
1
> 9.5 g/dL
0
growth retardation
present
2
absent
0
thalassemia facies
definite
2
mild
1
absent
0
age at first blood transfusion
< 6 years
3
6 to 15 years
2
> 15 years
1
none
0
frequency of blood transfusion
>= 2 per years
2
<= 1 per year (occasional)
1
none
0
total score =
= SUM(points for all 6 parameters)
Interpretation:
• minimum score: 0
• maximum score: 12
• The higher the score the more severe the disease.
Score
Group
Severity
0 to 2
I
mild
3 to 6
II
moderate
7 to 13
III
severe
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