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Mahidol Score of Sripichai et al for beta-Thalassemia/Hemoglobin E Disease

Description

Sripichai et al developed a score for measuring the severity of beta-thalassemia.hemoglobin E disease. This can help to identify a patient who may benefit from more aggressive management. The authors are from Mahidol University, Chulalongkorn University, Ramathibodi Hospital and Nakhonpathom Hospital in Thailand.


Patient selection: beta-thalassemia/hemoglobin E, pediatric and young adult

 

Parameters:

(1) hemoglobin at steady state in g/dL

(2) age of first blood transfusion

(3) requirement for blood transfusion

(4) size of spleen in centimeters

(5) age at thalassemia presentation

(6) growth and development

 

Parameter

Finding

Points

hemoglobin at steady state

> 7.5 g/dL

0

 

6 to 7.5 g/dL

1

 

< 6 g/dL

2

age of first blood transfusion

> 10 years

0

 

5 to 10 years

1

 

< 5 years

2

requirements for blood transfusion

none or rare

0

 

occasional

1

 

regular

2

splenic size

< 3 cm

0

 

3 to 10 cm

1

 

> 10 cm

2

 

splenectomized

2

age at thalassemia presentation

> 10 years

0

 

3 to 10 years

0.5

 

< 3 years

1

growth and development

> 25th percentile

0

 

3rd to 25th percentile

0.5

 

< 3rd percentile

1

 

where:

• What the splenic measurement represents is unspecified. It may be distance below the left costal margin.

 

total score =

= SUM(points for all 6 parameters)

 

Interpretation:

• minimum score: 0

• maximum score: 10

• The higher the score the more severe the thalassemia.

 

Total Score

Severity

0 to 3.5

mild

4 to 7

moderate

7.5 to 10

severe

 


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