Acquired haemophilia A is a rare condition defined by the presence of coagulation inhibitors, which are autoantibodies directed against factor VIII that interfere with its activity. We report a case of a 69-year-old woman that presented with knee haemarthrosis followed by spontaneous retroperitoneal haematoma. On coagulation studies, she presented normal prothrombin time with prolonged activated partial thromboplastin time non-correcting on mixture test, low titers of factor VIII and was detected factor VIII inhibitor that led to diagnosis of acquired haemophilia A. She was managed with supportive measures to control haemorrhage and immunosuppressive therapy to eradicate inhibitors, initially with corticosteroids, with partial transitory response, after which she developed new spontaneous haematomas. Rituximab was started at that time, with a good outcome. The additional aetiological study identified autoimmune thyroiditis and autoimmune pangastritis, an association rarely described in literature.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8995960PMC
http://dx.doi.org/10.1136/bcr-2021-248701DOI Listing

Publication Analysis

Top Keywords

acquired haemophilia
12
factor viii
12
autoimmune thyroiditis
8
haemophilia associated
4
associated autoimmune
4
thyroiditis pangastritis
4
pangastritis acquired
4
haemophilia rare
4
rare condition
4
condition defined
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!