Retrospective evaluation of bleeding tendency and simultaneous thrombin and plasmin generation in patients with rare bleeding disorders

Rare bleeding disorders (RBDs) are a heterogeneous group of diseases with varying bleeding tendency, only partially explained by their laboratory phenotype. We analysed the separate groups of RBD abnormalities, and we investigated retrospectively whether the novel haemostasis assay (NHA) was able to...

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Bibliographic Details
Published in:Haemophilia : the official journal of the World Federation of Hemophilia 2012-07, Vol.18 (4), p.630-638
Main Authors: Van GEFFEN, M., MENEGATTI, M., LOOF, A., LAP, P., KARIMI, M., LAROS-van GORKOM, B. A. P., BRONS, P., Van HEERDE, W. L.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
bleeding tendency
Blood Coagulation Disorders - complications
Blood Coagulation Disorders - metabolism
Cohort Studies
Female
Fibrinolysin - metabolism
Hemorrhage - etiology
Humans
Male
Middle Aged
phenotype
plasmin
Predictive Value of Tests
rare bleeding disorders
Retrospective Studies
thrombin
Thrombin - metabolism
Young Adult
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Summary:Rare bleeding disorders (RBDs) are a heterogeneous group of diseases with varying bleeding tendency, only partially explained by their laboratory phenotype. We analysed the separate groups of RBD abnormalities, and we investigated retrospectively whether the novel haemostasis assay (NHA) was able to differentiate between bleeding tendency. We have performed simultaneous thrombin generation (TG) and plasmin generation (PG) measurements in 41 patients affected with deficiencies in prothrombin, factor (F) V, FVII, FX, FXIII and fibrinogen. Parameters of the NHA were classified based on (major or minor) bleeding tendency. Patients with deficiencies in coagulation propagation (FII, FV and FX) and major type of bleedings had diminished TG (expressed as AUC) below 20% of control. FVII deficient patients only had prolonged thrombin lag‐time ratio of 1.6 ± 0.2 (P < 0.05) and normal AUC (92–125%). Afibrinogenemic patients demonstrated PG of 2–29% of normal and appeared to correlate with the type of mutation. Thrombin peak‐height (57 ± 16%) was reduced (not significant) in these patients and AUC was comparable to the reference (102 ± 27%). FXIII‐deficient plasmas resulted in a reduced thrombin peak‐height of 59 ± 13% (P 
ISSN:1351-8216
1365-2516
DOI:10.1111/j.1365-2516.2012.02759.x