Acquired haemophilia (AH) is a severe bleeding diathesis that affects both males and females. It is caused by suddenly appearing autoantibodies that interfere with coagulation factor VIII (FVIII) activity. Although some conditions such as autoimmune diseases, cancer and puerperium seem likely to induce AH, in more than half of the observed cases autoantibodies to FVIII are idiopathic. The clinical picture is characterized by spontaneous and post-traumatic subcutaneous bleeds as well as massive mucosal membrane hemorrhages (from the genitourinary and gastrointestinal tracts). Typical abnormalities in AH are prolonged activated partial thromboplastin time and normal results of the other haemostatic tests (platelet count, prothrombin and thrombin times, fibrinogen concentration). Acquired haemophilia is definitely confirmed by quantification of FVIII neutralizing antibodies. Bleeds are usually treated with activated prothrombin complex concentrates and activated recombinant factor VII. In most patients with AH, the use of immunosupressive agents results in autoantibody elimination and restoration of normal FVIII plasma activity.
Identification of hepatitis B virus (HBV) infection in the absence of surface antigen (HbsAg) became possible with the introduction of HBV DNA detection methods. Such occult HBV infection was diagnosed recently in about half of the Japanese HBsAg-negative haemophilia patients. The aim of our study was to assess the prevalence of occult HBV infection in Polish severe haemophilia population on the sample of 115 haemophilia A and B patients (mean age 34.9 +/- 10.9) treated with non-virus inactivated clotting factor preparations before 1995. HBV DNA was detected in nine HBsAg-positive patients (7.8%). The mean HBV DNA load was 72,800 IU mL(-1) (250-400,000 IU mL(-1)). Hepatitis C virus (HCV) RNA was found in six out of nine HBV-positive patients. In conclusion, HBV DNA was identified only in HBsAg-positive patients. Unlike in Japan, the frequency of occult HBV infection in Polish haemophilia population seems extremely rare or absent.
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