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External validation of the PLASMIC score: a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment
- Source :
- Journal of thrombosis and haemostasis : JTH. 16(1)
- Publication Year :
- 2017
-
Abstract
- Essentials Severe ADAMTS-13 deficiency is key to thrombotic thrombocytopenic purpura (TTP) diagnosis. PLASMIC score predicts ADAMTS-13 deficiency in suspected TTP with high discrimination. PLASMIC score is more generalizable with fewer missing data than alternative clinical scores. PLASMIC score identifies a subgroup of patients lacking significant response to plasma exchange. SummaryBackground The PLASMIC score was recently published to distinguish patients with severe ADAMTS-13 deficiency from those without for early identification of thrombotic thrombocytopenia purpura (TTP). Objective We performed an independent external validation of the PLASMIC score for clinical prediction of severe ADAMTS-13 deficiency. Patients/Methods We studied an independent cohort of 112 consecutive hospitalized patients with suspected thrombotic microangiopathy and appropriate ADAMTS-13 testing (including 21 patients with TTP diagnosis). Results The PLASMIC score model predicted severe ADAMTS-13 deficiency with a c statistic of 0.94 (0.88–0.98). When dichotomized at high (score 6–7) vs. low-intermediate risk (score 0–5), the model predicted severe ADAMTS-13 deficiency with positive predictive value of 72%, negative predictive value of 98%, sensitivity of 90% and specificity of 92%. In the low-intermediate risk group (score 0–5) there was no significant improvement in overall survival associated with plasma exchange. Conclusions The PLASMIC score model had excellent applicability, discrimination and calibration for predicting severe ADAMTS-13 deficiency. The clinical algorithm allowed identification of a subgroup of patients who lacked a significant response to empiric treatment.
- Subjects :
- Adult
Male
medicine.medical_specialty
Thrombotic microangiopathy
Thrombotic thrombocytopenic purpura
ADAMTS13 Protein
030204 cardiovascular system & hematology
Severity of Illness Index
Article
Decision Support Techniques
03 medical and health sciences
0302 clinical medicine
Risk groups
Predictive Value of Tests
Internal medicine
medicine
Humans
THROMBOCYTOPENIA PURPURA
Plasma Exchange
Purpura, Thrombotic Thrombocytopenic
business.industry
External validation
Reproducibility of Results
Hematology
Middle Aged
medicine.disease
Purpura
Treatment Outcome
030220 oncology & carcinogenesis
Cohort
cardiovascular system
Significant response
Female
medicine.symptom
business
Biomarkers
Subjects
Details
- ISSN :
- 15387836
- Volume :
- 16
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Journal of thrombosis and haemostasis : JTH
- Accession number :
- edsair.doi.dedup.....76a130e27f58d0114848e02e7e4850e2