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001-es BibID:BIBFORM072591
035-os BibID:(WoS)000431663100004 (Scopus)85044290673
Első szerző:Muszbek László (haematológus, kutató orvos)
Cím:Auto-, and alloantibodies against factor XIII : laboratory diagnosis and clinical consequences / Muszbek L., Pénzes K., Katona É.
Dátum:2018
ISSN:1538-7933
Megjegyzések:Acquired FXIII deficiencies caused by autoantibodies against FXIII subunits represent rare but very severe bleeding diatheses. Alloantibodies in FXIII deficient patients also cause life-threatening bleeding complication, but they develop extremely rarely. In this review we provide an overview on the diagnosis and classification of anti-FXIII antibodies and analyze 48 patients with autoimmune FXIII deficiency and 4 additional FXIII deficient patients who developed anti-FXIII alloantibody. The patients were collected from peer-reviewed publications from which relevant data could be extracted. With the exception of two cases the antibodies were directed against FXIII-A. The difficulties in the diagnosis of FXIII deficiency in the presence of anti-FXIII antibodies are discussed and a scheme for the functional classification of the anti-FXIII antibodies is recommended. The three main categories are neutralizing and non-neutralizing antibodies and antibodies with combined effect. The methods being used for detecting and quantifying the inhibitory effect on FXIII activation and on the transglutaminase activity of activated FXIII are summarized and techniques for the classification of neutralizing anti-FXIII antibodies are outlined. The importance of clearance studies in these cases is emphasized. Binding assays, useful for the identification of non-neutralizing and combined type antibodies, were collected from literature and their informative power is demonstrated by examples. The most frequently occurring bleeding symptoms in patients with anti-FXIII antibodies were soft tissue bleeding, intracranial bleedings also occurred, but less frequently than in inherited FXIII deficiency. Treatment of such patients is extremely challenging, the main aim should be eradication of the antibody. This article is protected by copyright. All rights reserved.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Journal Of Thrombosis And Haemostasis. - 16 : 5 (2018), p. 822-832. -
További szerzők:Pénzes-Daku Krisztina (1978-) (biológus) Katona Éva (1961-) (klinikai biokémikus)
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