Krvácivé stavy představují situace, které se klinicky projevují krvácením nebo jeho zvýšeným rizikem. Důvodů je celá řada. Vedle anatomických příčin patří mezi hlavní důvody hemoragie porucha hemostázy. Z klinického pohledu jde o situace, kdy je krvácení neúměrné vyvolávajícímu podnětu nebo vzniká spontánně, mohou se také projevovat obtížně stavitelným nebo protrahovaným krvácením po operaci nebo poranění. Podkladem je narušení rovnováhy v důsledku kvantitativní či kvalitativní poruchy některého z hemostatických mechanismů, příčiny se mohou i kombinovat. Cílem příspěvku je poskytnout, ve vymezeném rozsahu, přehled těchto stavů se zdůrazněním aspektů využitelných v běžné klinické praxi.
bleeding disorders are situations manifested clinically by bleeding or an increased risk of bleeding caused by various reasons. In addition to anatomical causes, the main reasons for haemorrhage include impaired haemostasis. From the clinical point of view these are clinical situations in which bleeding is inadequate to inducing stimulus or begins spontaneously and may manifested itself as difficult-to-stop bleeding or prolonged bleeding after operation or after injury. The basis is disturbance of balance due to a quantitative or qualitative disturbance of one of the hemostatic mechanisms, the causes can be combined. The aim of this article is to provide an overview of these conditions in defined scope, highlighting aspects useful in routine clinical practice.
- MeSH
- diseminovaná intravaskulární koagulace diagnóza patofyziologie terapie MeSH
- hemofilie A diagnóza patofyziologie terapie MeSH
- homeostáza MeSH
- krvácení * diagnóza etiologie terapie MeSH
- lidé MeSH
- trombocytopatie diagnóza patofyziologie terapie MeSH
- trombotická trombocytopenická purpura diagnóza patofyziologie terapie MeSH
- von Willebrandova nemoc diagnóza patofyziologie terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
The aim of this study was to determine the thrombogenicity of lupus anticoagulant (LA) antibodies using a modified thrombin generation assay (TGA) with the addition of activated protein C (APC) in a group of 85 patients with LA-positive samples. Of these, 58 patients had clinical manifestations of antiphospholipid syndrome (APS) according to the Sydney criteria classification, i.e., each patient had thrombosis or foetal loss, and 27 patients did not show any clinical manifestations of APS. A comparison of the two groups' TGA results revealed statistically significant differences (Fisher's test p = 0.0016). The group of patients exhibiting clinical manifestations of APS showed higher thrombogenicity in 56.9% of patients, while the group of patients not yet exhibiting clinical manifestations of APS showed higher thrombogenicity in 25.9% of patients. There were no significant differences in the specificity of the TGA test between the groups of patients exhibiting similar clinical manifestations. Receiver operating characteristic curve analysis showed a more significant relationship (p = 0.0060) for TGA than for LA titre (p = 0.3387). These data suggest that the determination of LA thrombogenicity with the TGA assay leads to an increased prediction of the manifestation of a thromboembolic event. Our findings appear to be particularly relevant for the prediction of thrombotic events in patients with laboratory-expressed APS and no clinical manifestations.
- Publikační typ
- časopisecké články MeSH
Antiphospholipid syndrome (APS) is a hypercoagulable state accompanied by the presence of heterogeneous antiphospholipid antibodies (aPL), which nonspecifically affect hemostasis by the presence of lupus anticoagulans (LA), anticardiolipin antibodies (aCL), antibodies against β2-glycoprotein-I (anti-β2GPI), but also non-criteria antibodies such as antibodies against β2-glycoprotein-I domain I (anti-DI), anti-phosphatidylserine/prothrombin (anti-PS/PT), anti-annexin V, and many others. The main target of the antibodies is the activated protein C (APC) system, the elimination of which can manifest itself as a thrombotic complication. The aim of this study was to determine the thrombogenicity of antibodies using a modified protein C-activated thrombin generation assay (TGA) on a group of 175 samples suspected of APS. TGA was measured with/without APC and the ratio of both measurements was evaluated (as for APC resistance), where a cut-off was calculated ≤4.5 (90th percentile) using 21 patients with heterozygous factor V Leiden mutation (FV Leiden heterozygous). Our study demonstrates the well-known fact that multiple positivity of different aPLs is a more severe risk for thrombosis than single positivity. Of the single antibody positivity, LA antibodies are the most serious (p value < 0.01), followed by aCL and their subgroup anti-DI (p value < 0.05). Non-criteria antibodies anti-annexin V and anti-PT/PS has a similar frequency occurrence of thrombogenicity as LA antibodies but without statistical significance or anti-β2GPI1 positivity. The modified TGA test can help us identify patients in all groups who are also at risk for recurrent thrombotic and pregnancy complications; thus, long-term prophylactic treatment is appropriate. For this reason, it is proving increasingly beneficial to include the determination antibodies in combination with modified TGA test.
- MeSH
- antifosfolipidové protilátky MeSH
- antifosfolipidový syndrom * komplikace MeSH
- antikardiolipinové protilátky MeSH
- beta-2-glykoprotein I MeSH
- fosfatidylseriny MeSH
- lidé MeSH
- protein C MeSH
- protrombin MeSH
- těhotenství MeSH
- thrombin MeSH
- trombóza * etiologie MeSH
- Check Tag
- lidé MeSH
- těhotenství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Publikační typ
- abstrakt z konference MeSH
- MeSH
- hematologie * MeSH
- kongresy jako téma MeSH
- Publikační typ
- zprávy MeSH
