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

Description

Specialty