[Prevalence of HCV, HBV and HIV infections among severe Polish haemophiliacs]. / Czestosc zakazen HCV, HBV i HIV u chorych na ciezka hemofilie w Polsce: porównanie chorych urodzonych przed i po 1991 roku.

UNLABELLED: In the past, haemophilia replacement therapy was based on fresh frozen plasma, cryoprecipitate and blood transfusions--i.e. preparates not subjected to any viral inactivation methods. The aim of the present study was to assess the prevalence of HCV, HBV and HIV infections among Polish haemophiliacs. MATERIALS AND METHODS: Severe haemophilia patients were divided into two age groups according to the type of replacement therapy used in the past: group 1--172 patients born 1935-1990, group 2--41 patients born 1991-2002. The following viral markers were tested: anti-HCV, RNA HCV (if needed--virus genotype), HBsAg and anti-HIV. In 75 patients / group 1 and 41 / group 2, anti-HBc presence was determined. RESULTS: In group 1, 95% of patients were anti-HCV positive and in 77.3% RNA HCV was detected. The most prevalent was lb genotype (58.6%) followed by 3a (27%). HBsAg was found in 8.7% cases of group 1. Anti-HCV antibodies were detected in 51 of 75 (68%) patients of this group. One patient was anti-HIV positive. In only one patient of group 2 (2.4%) viral markers were detected (anti-HCV and RNA HCV). CONCLUSION: Nowadays, the risk of HCV, HBV and HIV infection in Polish severe haemophiliacs is very low.

[Acquired haemophilia]. / Hemofilia nabyta.

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 immunosuppressive agents results in autoantibody elimination and restoration of normal FVIII plasma activity.