Startseite Development and validation of a multivariable prediction rule for detecting a severe acquired ADAMTS13 activity deficiency in patients with thrombotic microangiopathies
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Development and validation of a multivariable prediction rule for detecting a severe acquired ADAMTS13 activity deficiency in patients with thrombotic microangiopathies

  • Jorge M. Nieto ORCID logo EMAIL logo , Félix De La Fuente-Gonzalo ORCID logo , Fernando A. González , Ana Villegas , Rafael Martínez , Manuel E. Fuentes und Paloma Ropero
Veröffentlicht/Copyright: 10. August 2017
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Clinical Chemistry and Laboratory Medicine (CCLM)
Aus der Zeitschrift Clinical Chemistry and Laboratory Medicine (CCLM) Band 56 Heft 2

Abstract

Background:

Thrombotic microangiopathies (TMAs) are a group of diseases that have different aetiologies and treatments, but a clinical differential diagnosis remains difficult. Among TMAs, thrombotic thrombocytopenic purpura (TTP) is characterised by a severe ADAMTS13 functional deficiency. However, assays exploring ADAMTS13 activity are limited to some specialised laboratories. Our objective was to develop and validate a diagnostic method for TTP in adult patients with TMA.

Methods:

We generated a multivariable model (four predictors) on a cohort of 174 TMA patients in order to predict an ADAMTS13 activity deficiency (AUC of 0.927). The multivariable model was simplified into a binary rule to facilitate the interpretation of the predictions. There were two scenarios for a patient: (1) Predicted ADAMTS13 deficiency; if the patient met four conditions simultaneously (platelets ≤44×109/L, creatinine ≤2 mg/dL (≤176.84 µmol/L) for males or ≤1.9 mg/dL (≤168 µmol/L) for females, age ≤68 years and no history of haematopoietic stem cell transplant [HSCT]); or (2) Predicted “normal” activity; if any of the above conditions are not met. This rule was validated on a second cohort of 86 patients and performed with sensitivity of 87.7% and specificity of 92.7%.

Results and conclusions:

This could lead to the earlier confirmation or rapid exclusion of TTP when ADAMTS13 testing is not avalilable, facilitating a more suitable therapy based on the aetiology of the TMA.

Keywords: ADAMTS13; diagnostic rule; prediction model; thrombotic microangiopathies; thrombotic thrombocytopenic purpura

  1. Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

  2. Research funding: None declared.

  3. Employment or leadership: None declared.

  4. Honorarium: None declared.

  5. Competing interests: Jorge M. Nieto, Ana Villegas, Fernando A. González and Félix De La Fuente-Gonzalo have received fees for acting as speakers for Alexion Pharmaceuticals Inc. Paloma Ropero, Manuel E. Ferrer and Rafael Martínez declare no conflict of interest. The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.

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Supplemental Material:

The online version of this article offers supplementary material (https://doi.org/10.1515/cclm-2017-0437).

Received: 2017-05-17
Accepted: 2017-07-05
Published Online: 2017-08-10
Published in Print: 2018-01-26

©2018 Walter de Gruyter GmbH, Berlin/Boston

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https://doi.org/10.1515/cclm-2017-0437
Schlagwörter für diesen Artikel
ADAMTS13; diagnostic rule; prediction model; thrombotic microangiopathies; thrombotic thrombocytopenic purpura

Artikel in diesem Heft

  1. Frontmatter
  2. Editorial
  3. Need for better PTH assays for clinical research and patient treatment
  4. Reviews
  5. Liquid biopsy in ovarian cancer: recent advances on circulating tumor cells and circulating tumor DNA
  6. Hair testing of GHB: an everlasting issue in forensic toxicology
  7. Opinion Paper
  8. The use of error and uncertainty methods in the medical laboratory
  9. Genetics and Molecular Diagnostics
  10. Results of the first external quality assessment scheme (EQA) for isolation and analysis of circulating tumour DNA (ctDNA)
  11. The importance of biochemical and genetic findings in the diagnosis of atypical Norrie disease
  12. General Clinical Chemistry and Laboratory Medicine
  13. Various glycolysis inhibitor-containing tubes for glucose measurement cannot be used interchangeably due to clinically unacceptable biases between them
  14. Measurement uncertainty of γ-glutamyltransferase (GGT) in human serum by four approaches using different quality assessment data
  15. Oxidation of PTH: in vivo feature or effect of preanalytical conditions?
  16. A comparison between high resolution serum protein electrophoresis and screening immunofixation for the detection of monoclonal gammopathies in serum
  17. The key incident monitoring and management system – history and role in quality improvement
  18. Is it necessary for all samples to quantify 25OHD2 and 25OHD3 using LC-MS/MS in clinical practice?
  19. Contamination of dried blood spots – an underestimated risk in newborn screening
  20. Comparative study of the diagnostic and prognostic value of antibodies against chimeric citrullinated synthetic peptides and CCP3/CCP3.1 assays
  21. Development and validation of a multivariable prediction rule for detecting a severe acquired ADAMTS13 activity deficiency in patients with thrombotic microangiopathies
  22. Use of the sFlt-1/PlGF ratio to rule out preeclampsia requiring delivery in women with suspected disease. Is the evidence reproducible?
  23. Evaluation of a new free light chain ELISA assay: bringing coherence with electrophoretic methods
  24. Hematology and Coagulation
  25. The frequency of occurrence of fish-shaped red blood cells in different haematologic disorders
  26. Reference Values and Biological Variations
  27. Pediatric reference intervals for 29 Ortho VITROS 5600 immunoassays using the CALIPER cohort of healthy children and adolescents
  28. Cancer Diagnostics
  29. Prevalence and causes of abnormal PSA recovery
  30. Cardiovascular Diseases
  31. Serum omentin-1 is a novel biomarker for predicting the functional outcome of acute ischemic stroke patients
  32. Letters to the Editor
  33. BD Vacutainer® Barricor tube in the emergency department: reduced hemolysis rates using partial draw tubes with reduced vacuum
  34. The risk of unjustified BRCA testing after the “Angelina Jolie effect”: how can we save (laboratory) medicine from the Internet?
  35. Sweat travels: the issue of sweat chloride transportation
  36. Individual values of antineutrophil cytoplasmic antibodies do not correspond between antigen-specific assays
  37. False negative results caused by erroneous automated result interpretation algorithm on the FilmArray 2.0 instrument
  38. Quantification of 1,25-dihydroxyvitamin D – value of manufacturers’ product information
  39. Interference between ethosuximide and barbiturates in an immunochromatographic method
  40. In vivo interference of Ioversol in serum and urine capillary electrophoresis: an optimized protocol for sample collection
  41. Evaluation of the new Elecsys SCC assay: comparison with the Kryptor SCC assay
  42. Congress Abstracts
  43. 9th National Congress of the Portuguese Society of Clinical Chemistry, Genetics and Laboratory Medicine
  44. ACBI 2017 40TH Annual Conference
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