[Acquired haemophilia B: a case report and literature review].

Abstract

Acquired haemophilia is a rare disease; it occurs most frequently in elderly patients. The majority of cases are due to autoantibodies to factor VIII, which deplete circulating factor VIII or acquired haemophilia A. Only few cases of acquired haemophilia B are reported until today. We report a case of a 7-year-old girl with no past medical history of bleeding disorder and who present an extensive haematoma in the left calf. The diagnosis was established by the demonstration of an isolated prolongation of the activated partial thromboplastin time (APTT) with a reduced factor IX level and evidence of factor IX inhibitor activity to 2 Bethesda Unit (2UB). Diagnosis of acquired haemophilia B confirmed, patient received recombinant factor VIIa and corticosteroid treatment. Bleeding symptoms had completely disappeared and coagulation tests become normal. In conclusion, if bleeding symptoms are associated with unexplained prolongation of APTT, an inhibitor against factor must be searched for not missing an acquired coagulation disease.

• Authors: Inès Jedidi, Sondes Hdiji, Naourez Ajmi, Faiza Makni, Sayda Masmoudi, Moez Elloumi, Choumous Kallel
• Journal: Annales de biologie clinique (Ann Biol Clin (Paris)) 2011 Nov-Dec Vol. 69 Issue 6 Pg. 685-8 ISSN: 0003-3898 [Print] France
• Vernacular Title: Hémophilie B acquise : à propos d'un cas avec revue de la littérature.
• PMID: 22123568 (Publication Type: Case Reports, English Abstract, Journal Article, Review)

Chemical References

• Autoantibodies
• Factor IX

Topics

• Autoantibodies (adverse effects)
• Child
• Factor IX (antagonists & inhibitors, immunology)
• Female
• Hematoma (blood, diagnosis)
• Hemophilia B (diagnosis, etiology)
• Humans
• Partial Thromboplastin Time