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Model of Benhamou et al for Predicting Mortality in a Patient with Acquired ADAMTS13-Deficient Idiopathic Thrombotic Thrombocytopenia Purpura (TTP)

Description

Benhamou et al evaluated patients with acquired thrombotic thrombocytopenic purpura (TTP). A number of risk factors can help to identifiy a patient at increased risk for mortality who may benefit from more aggressive management. The authors are from multiple institutions in France.


Patient selection: idiopathic thrombotic thrombocytopenic purpura (TTP) with acquired ADAMTS13 defiiciency

 

Outcome: 30-day mortality

 

Parameters:

(1) age of the patient in years

(2) cerebral involvement

(3) serum LDH concentration relative to the upper limit of the normal reference range (ULN)

 

ratio of serum LDH to ULN =

= (serum LDH in IU/L) / (upper limit of normal for normal reference range in IU/L)

 

Parameter

Finding

Points

age in years

<= 40 years

0

 

41 to 60 years

1

 

> 60 years

2

cerebral involvement

absent

0

 

present

1

LDH ratio

< 10

0

 

>= 10

1

 

total score =

= SUM(points for all 3 parameters)

 

Interpretation:

• minimum score: 0

• maximum score: 4

• The higher the score the greater the mortality rate.

 

Total Score

Risk Group

0 or 1

low

2

intermediate

3 or 4

high

 

 

Score

Mortality Rate

0

0 to 3%

1

5 to 13%

2

11 to 27%

3

33 to 39%

4

66%

from derivation and validation cohorts

 

Performance:

• The area under the ROC curve was 0.77.


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