Abstract Number: PB0777
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Acquired Hemorrhagic Coagulation Disorders
Background: Acquired Hemophilia A (AHA) is a rare entity, resulting from the production of autoantibodies against factor VIII of the coagulation process. The presence of these autoantibodies, usually idiopathic, may be related to autoimmune conditions, drugs, neoplastic diseases and pregnancy. The diagnosis involves clinical aspects and laboratorial findings, such as prolonged activated partial thromboplastin time (aPTT), decreased levels of factor VIII (FVIII) and the presence of inhibitors against it. Clinical manifestations of AHA are characterized by mucous-cutaneous, intramuscular and/or postpartum bleeding.
Aims: To present a case of AHA associated with pregnancy with several muco-cutaneous hemorrhagic manifestations and intramuscular hematomas with excellent response to the use of immunosuppressants.
Methods: A 34-years-old female underwent cesarean delivery without complications. Forty days later she developed spontaneous hematomas with progressive worsening and intramuscular bleedings. She had no personal/family history of hematologic or autoimmune disease. Referred to hematological investigation at Fundação Hemominas Juiz de Fora-Brazil. The laboratory screening tests for coagulation disorders showed an increase of aPTT by its own, reduction of FVIII levels and the presence of inhibitors against it. Tests for diagnosis of autoimmune diseases and screening for neoplasms were negative. AHA was diagnosed. The patient was treated with prednisone plus tranexamic acid and showed total improvement, normalization of serum levels of Factor VIII, without the presence of inhibitors.
Results: Laboratory study: aPTT 62.5”, FVIII coagulation assay 3.5%, FVIII inhibitors 2.5 Bethesda. Diagnosis was confirmed and the instituted therapy was satisfactory.
Conclusions: Although rare, it is important to consider the diagnosis of AHA for cases of puerperal bleeding in which the treatment based on its most common causes is unsatisfactory. To improve the forecast, an early diagnosis is fundamental and it is essential to disseminate knowledge about this entity among the medical professionals.
To cite this abstract in AMA style:
O W Rodrigues D, F Santos O, N S Magalhaes N, L Medeiros R, Almeida RDM, A S Lopes J, O F Junior I, O W Rodrigues L. Postpartum Acquired Hemophilia A Persists as a Diagnostic Challenge [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/postpartum-acquired-hemophilia-a-persists-as-a-diagnostic-challenge/. Accessed October 2, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/postpartum-acquired-hemophilia-a-persists-as-a-diagnostic-challenge/