Acquired haemophilia A associated to autoimmune thyroiditis and pangastritis

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...

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Bibliographic Details
Published in:BMJ case reports 2022-04, Vol.15 (4), p.e248701
Main Authors: Marques Dias, Joana Isabel, Ferreira, Marta Azevedo, Grilo, Ana, Reis, Ana Isabel
Format: Article
Language:English
Subjects:
Abdomen
Aged
Anemia
Anticoagulants
Autoantibodies
Autoimmune diseases
Case reports
Case Reports: Unusual association of diseases/symptoms
Drugs
Factor VIII
Female
Hashimoto Disease - complications
Hematoma - complications
Hemoglobin
Hemophilia
Hemophilia A - complications
Hemophilia A - diagnosis
Hemorrhage - etiology
Humans
Immunotherapy
Internal medicine
Laboratories
Medical prognosis
Monoclonal antibodies
Mortality
Remission (Medicine)
Thyroid gland
Thyroiditis, Autoimmune - complications
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Summary: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.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2021-248701