Anti-factor XIII A subunit (FXIII-A) autoantibodies block FXIII-A2 B2 assembly and steal FXIII-A from native FXIII-A2 B2
نویسندگان
چکیده
منابع مشابه
Molecular mechanism of a mild phenotype in coagulation factor XIII (FXIII) deficiency: a splicing mutation permitting partial correct splicing of FXIII A-subunit mRNA.
Congenital factor XIII (FXIII) deficiency is potentially a severe bleeding disorder, but in some cases, the symptoms may be fairly mild. In this study, we have characterized the molecular mechanism of a mild phenotype of FXIII A-subunit deficiency in a Finnish family with two affected sisters, one of whom has even had two successful pregnancies without regular substitution therapy. In the scree...
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doi:10.1160/TH11-09-0625 Thromb Haemost 2012; 107: 592–594 Dir Sirs, Factor XIII (FXIII) is a fibrin-stabilising factor which crosslinks fibrin monomers among themselves as well as to α2-plasmin inhibitor and fibronectin, and thus contributes to haemostasis, wound healing, and maintenance of pregnancy (1–3). Congenital FXIII deficiency is a rare haemorrhagic disorder. Umbilical bleeding in the ...
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light of the results of the French study, one can only agree with the recommendation of Lepretre et al that FCA should not be used for the treatment of CLL outside of clinical trials. The question of whether alemtuzumab should no longer be used in any chemoimmu-notherapy is an entirely different issue. It should be emphasized that a recent random-ized trial showed that the second-line use of fl...
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UNLABELLED Inherited mild factor XIII deficiency belongs to one of the most underdiagnosed bleeding disorders so far. This is, because most patients do not develop bleeding complications in daily life. Patient, methods: A man (age: 64 years) without a history of bleeding presented with painful swelling of neck, weight loss, anemia and episodic bleeding from the right tonsil necessitating tonsil...
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INTRODUCTION Abdominal aortic aneurysms (AAA) are characterized by a progressive dilatation of the abdominal aorta, and are associated with a high risk of rupture once the dilatation exceeds 55 mm in diameter. A large proportion of AAA develops an intraluminal thrombus, which contributes to hypoxia, inflammation and tissue degradation. We have previously shown that patients with AAA produce clo...
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ژورنال
عنوان ژورنال: Journal of Thrombosis and Haemostasis
سال: 2015
ISSN: 1538-7933
DOI: 10.1111/jth.12877