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Factor XIII - an under diagnosed deficiency - are we using the right assays?

Lawrie, AS; Green, L; Mackie, IJ; Liesner, R; Machin, SJ; Peyvandi, F; (2010) Factor XIII - an under diagnosed deficiency - are we using the right assays? J THROMB HAEMOST , 8 (11) 2478 - 2482. 10.1111/j.1538-7836.2010.04028.x.

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Abstract

Background: The clot solubility test is the most widely used method for detection of factor (F)XIII deficiency. However, it will only detect severe deficiencies; consequently mild deficiencies and heterozygous states are probably under diagnosed. Objective: As an alternative first-line screening test, we assessed an automated quantitative ammonia release assay (QARA). Patients/methods: Inter-assay imprecision was evaluated with commercial normal and pathological control plasmas (10 replicates on each of 5 days). Using the QARA and other commercial assays a comparative assessment of congenital (FXIII range < 1-70 u dL-1, n = 9) and acquired (n = 43) deficiencies was made. We also investigated the prevalence of acquired deficiencies in hospitalized patients using residual samples from adult patients (n = 1004) and from a paediatric intensive care unit (ICU, n = 56). Results: Assay imprecision was acceptably low (normal control: mean 86.6 u dL-1; cv = 2.0%; pathological control: mean 27.5 u dL-1; cv = 3.8%). Using an iodoacetamide blanking procedure, the QARA results (FXIII range < 1-70 u dL-1) exhibited close agreement with those from an immuno-turbidometric FXIII A-subunit (FXIII-A) method. There was also good correlation (R2 >= 0.89) between the QARA (range 20-180 u dL-1), a second chromogenic assay, the FXIII-A and FXIII A+B-subunit ELISA. We found that 21% of samples from adult patients had FXIII levels < 70 u dL-1 (mean normal +/- 2 SD 73-161 u dL-1) with 6% < 50 u dL-1. Within the paediatric ICU samples, 52% were < 70 u dL-1, with 21% < 50 u dL-1. Conclusions: Our data demonstrates that the automated assay is sensitive, highly reproducible and the results from clinical samples suggest that acquired FXIII deficiency is a relatively common phenomenon in hospital patients after surgery and in ICU.

Type: Article
Title: Factor XIII - an under diagnosed deficiency - are we using the right assays?
DOI: 10.1111/j.1538-7836.2010.04028.x
Keywords: acquired FXIII deficiency, factor XIII, factor XIII deficiency, FXIII assay, COAGULATION-FACTOR-XIII, PLASMA FACTOR-XIII, RARE BLEEDING DISORDERS, PHOTOMETRIC ASSAY, FIBRIN, SYSTEM, ANGIOGENESIS, MANAGEMENT, ACTIVATION, THROMBOSIS
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Haematology
URI: http://discovery.ucl.ac.uk/id/eprint/137254
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