Získaná hemofilie typu A – vzácná komplikace primárního Sjögrenova syndromu
Kazuistiky a literární přehled
Authors:
J. Tomasová Studýnková
Authors‘ workplace:
Revmatologický ústav, Praha
Published in:
Čes. Revmatol., 26, 2018, No. 2, p. 64-70.
Category:
Overview
These case reports describe two cases of severe bleeding in a 65-year-old male and a 70-year-old female with primary Sjögren's syndrome (SjS).
Sjögren's syndrome is a systemic autoimmune disease affecting the exocrine glands which can be also manifested by other systemic symptoms.
Bleeding in these patients was mediated by a reduced function of the procoagulant component, factor VIII (FVIII) due to the presence of FVIII inhibitory autoantibodies – i.e. acute acquired haemophilia A (AHA). Because of the severity of AHA manifestations, rapid diagnosis and timely initiation of therapy are required. The treatment aims to resolve bleeding and to eradicate the inhibitor by means of immunosuppression.
Key words:
Acquired haemophilia A, bleeding, factor VIII inhibitor, Sjögren's syndrom
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