Ann Lab Med.  2023 Sep;43(5):485-492. 10.3343/alm.2023.43.5.485.

Performance Validation of Three Scoring Systems for the Prediction of Thrombotic Microangiopathy Due to Severe ADAMTS13 Deficiency and the Response to Therapeutic Plasma Exchange: First Study in Korea

• Park SH
• Kim HK
• Jeong J
• Lee SH
• Lee YJ
• Kim YJ
• Jo JC
• Lim JH

• KMID: 2551997
• DOI: http://doi.org/10.3343/alm.2023.43.5.485

Abstract

Background
The BENTLEY score (B-S), French thrombotic microangiopathy (TMA) Reference Center score (FTMA-S), and PLASMIC score (PLASMIC-S) have been developed for TMA diagnostic prediction. We retrospectively validated their predictive performances in patients with severe (<10%) disintegrin and metalloprotease with thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency in terms of the risk of TMA and response to therapeutic plasma exchange (TPE).
Methods
The predictive performances of the three scoring systems were compared in 145 patients with suspected TMA who underwent ADAMTS13 activity tests between January 2014 and September 2022. The response to TPE and mortality in TMA-positive patients were compared after risk stratification, using the Mann–Whitney U and Fisher’s exact tests.
Results
The PLASMIC-S, FTMA-S, and B-S showed area under the curve values of 0.820, 0.636, and 0.513, respectively, for predicting TMA positivity in high-risk patients. The PLASMIC-S showed higher sensitivity (81.8%), negative predictive value (91.2%), positive predictive value (PPV; 66.7%), and accuracy (82.1%) than the FTMA-S (72.7%, 82.1%, 41.0%, and 60.0%, respectively) and B-S (4.6%, 70.2%, 50.0%, and 69.7%, respectively). The PLASMIC-S also showed higher specificity than the FTMA-S (82.2% vs. 54.5%). The modified PLASMIC-S, including lactate dehydrogenase/upper limit of normal ratios, increased the specificity, PPV, and accuracy to 97.0%, 92.3%, and 92.4%, respectively. In TMA-positive patients, high risk assessed by the PLASMIC-S predicted higher platelet recovery rates and less TPE sessions required for recovery than for those assessed at low-to-intermediate risk.
Conclusions
PLASMIC-S is the preferred scoring system for detecting patients with TMA positivity and for prognosis before confirmation of ADAMTS13 activity.

Keyword

ADAMTS13; Scoring systems; Performance; Prediction; Thrombotic microangiopathy; Therapeutic plasma exchange

Figure

• 

  Fig. 1 ROC analysis of the three scoring systems, LDH/ULN ratio, and modified PLASMIC-S for the prediction of patients with TMA due to severe ADAMTS13 deficiency assessed to be at high risk. (A) ROC curves (left) and comparative analysis (right) of the three scoring systems. (B) ROC curves (left) and comparative analysis (right) of the LDH/ULN ratio, PLASMIC-S, and modified PLASMIC-S. Abbreviations: AUC, area under the curve; CI, confidence interval; LDH, lactate dehydrogenase; ULN, upper limit of normal (230 IU/L in our study); PLASMIC-S, PLASMIC score; TMA, thrombotic microangiopathy; ADAMTS13, a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13.

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