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Disease Severity Scoring System (DSSS) of Cappellini et al for Non-Transfusion-Dependent Thalassemia (NTDT): Hematologic and Iron Status

Description

Cappellini et al for developed a Disease Severity Scoring System (DSSS) for patients with non-transfusion-dependent thalassemia (NTDT). One component deals with hematologic and iron status.The authors are from the University of Milan, University College London, American University of Beirut, University of Athens, Mahidol University (Bangkok) and the University of Cagliari.


 

Patient selection: non-transfusion-dependent thalassemia (NTDT)

 

Parameters:

(1) hemoglobin in g/dL (current or from any value within the past 3 months)

(2) platelet count per microliter (current or from any value within the past 3 months)

(3) iron status (serum ferritin in ng/mL OR liver iron content in mg iron per gram dry weight with precedent give to liver iron content), current or recent (within 3 months for serum ferritin, within 6 months for liver iron content)

(4) historical iron status if iron status scored 0 to 3

 

Parameter

Finding

Points

hemoglobin

> 10 g/dL

0

 

7.01 to 10 g/dL

1

 

5.01 to 7 g/dL

2

 

<= 5 g/dL

3

platelet count

> 400,000 per µL

2

 

150,000 to 400,000 per µL

0

 

< 150,000 per µL

1

iron status

liver iron <= 3 mg Fe per gram dry wgt

0

 

liver iron 3.01 to 5 mg Fe per gram dry wgt

1

 

liver iron 5.01 to 7 mg Fe per gram dry wgt

2

 

liver iron 7.01 to 15 mg Fe per gram dry wgt

3

 

liver iron > 15 mg Fe per gram dry wgt

4

 

serum ferritin <= 300 ng/mL

0

 

serum ferritin 301 to 800 ng/mL

1

 

serum ferritin 801 to 1500 ng/mL

2

 

serum ferritin 1501 to 2500 ng/mL

3

 

serum ferritin > 2500 ng/mL

4

historical status (if iron status <= 3)

liver iron > 15 mg Fe per gram dry wgt OR serum ferritin > 2500 ng/mL

1

 

liver iron <= 15 mg Fe per gram dry wgt AND serum ferritin <= 2500 ng/mL

0

 

 

subscore for hematologic and iron status =

= SUM(points for all 4 parameters)

 

Interpretation:

• minimum subscore: 0

• maximum subscore: 9

 


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