J Korean Med Sci.  2011 Sep;26(9):1247-1249. 10.3346/jkms.2011.26.9.1247.

The First Case of Postpartum Acquired Hemophilia A in Korea

• Lee JH
• Kim DH
• Yoo K
• Choi Y
• Kim SH
• Kim HJ

• KMID: 1779404
• DOI: http://doi.org/10.3346/jkms.2011.26.9.1247

Abstract

Acquired hemophilia A (AHA) is a rare coagulopathy caused by autoantibodies to coagulation factor VIII (FVIII). Most patients with AHA have been previously healthy; however, a variety of morbidities have been associated with the condition including pregnancy. A 40-yr-old woman visited our institution with extensive hematoma on the right hip area. Her medical history revealed no personal or familial history of bleeding diathesis. Her coagulation tests showed markedly prolonged aPTT (117 sec), markedly decreased level of FVIII activity (0.4%) and high-titer FVIII inhibitor (77 BU). Collectively, she was diagnosed as having postpartum AHA and was treated with bypassing agents and corticosteroids. Her aPTT was normalized on the 174th postpartum day and FVIII inhibitor showed negative conversion on the 224th postpartum day. This is the first case of postpartum AHA with high-titer FVIII inhibitor in Korea. Timely diagnosis and management can reduce morbidity and mortality of this potentially life-threatening condition.

Keyword

Acquired hemophilia A; Postpartum; Factor VIII inhibitor; Korea

MeSH Terms

Adrenal Cortex Hormones/therapeutic use
Adult
Autoantibodies/blood
Blood Coagulation Factors/therapeutic use
Factor VIII/immunology
Factor VIIa/therapeutic use
Female
Hematoma/diagnosis
Hemophilia A/*diagnosis/therapy
Humans
Partial Thromboplastin Time
Postpartum Period
Pregnancy
Recombinant Proteins/therapeutic use
Republic of Korea

Figure

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  Fig. 1 Clinical course of the present case of postpartum acquired hemophilia A.

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