Background: Acquired hemophilia A is a rare but potentially life-threatening bleeding disorder caused by auto-antibodies directed mostly against coagulation factor VIII (FVIII). Acquired hemophilia A usually occurring older adults, and occasionally post-partum. The appearance of FVIII antibodies may be associated with several underlying conditions such as autoimmune diseases, diabetes, malignancies and use of medications; however, its cause is undetermined (idiopathic) in up to 50% of reported cases.

Aims: A new case of acquired hemophilia A in a young nulliparous woman is reported.

Methods: A 18-year-old female was admitted to department of hematology with complaints of one week history of both arm ecchymosis. Family and personal history of bleeding diathesis were negative. Coagulation profile showed prolonged activated partial thromboplastin time (aPTT) of 84.5 seconds (reference range 26 – 42 seconds), which was not corrected via mixing with normal plasma. Results of coagulation test showed that factor IX (91.1%) was normal and FVIII activity was markedly decreased (2.1%), and FVIII inhibitor level was 20.16 Bethesda units (BU). Lupus anticoagulant was negative. Complete blood count was follows: hemoglobin 11.1 g/dL, white cell count 6.28 x 10⁹/L, platelet count 249 x 10⁹/L.

Results: The patient was treated with prednisolone, and FVIII inhibitor was not detected after 7 days. Three month later, she had a good response and stopped the medication.

Conclusions: Acquired hemophilia A in young age is extremely rare and its outcome seems more favorable than in older adults because the inhibitors usually resolve more quickly and in a higher rate of patients. However, due to acquired hemophilia has a significant mortality, it is important to be prompt recognition and treatment.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/acquired-hemophilia-a-in-young-nulliparous-woman-a-case-report/