Acquired hemophilia A is a rare but severe autoimmune bleeding disorder. It is more frequent in the elderly and results from the presence of autoantibodies directed against clotting factor VIII. In this review, we briefly report on the present state of knowledge regarding acquired hemophilia A, analyzing its epidemiology, pathogenesis, diagnostic, and clinical features. We also describe the main characteristics of this disorder according to its association with different conditions and the most important advances in the treatment of bleeding episodes and the eradication of the autoantibody. Am.

This recombinant concentrate is efficacious in FVII-deficient patients. It is safe since any risk of transmission of blood-borne viruses is eliminated.

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Background and Objectives: Factor VII (FVII) deficiency is a rare coagulation disorder, historically treated with prothrombin complex concentrates or plasma–derived FVII concentrates. We treated such patients (n = 17) with a recombinant, activated FVII preparation. Materials and Methods: Twenty–seven spontaneous bleeding episodes were treated and 7 major and 13 minor surgical interventions were carried out. The dosages employed ranged from 8.08 to 70.5 Ìg/kg body weight. Results: A mean dose between 22 and 26 Ìg/kg was sufficient to normalise the prothrombin time. Fifteen haemarthroses were treated with single doses and results were excellent in 13 cases. In 5/6 bleeding episodes of other types, the treatment gave either excellent or at least effective results. Haemostasis was secured in the 7 major and 13 minor surgical interventions. One patient developed antibodies 4–5 weeks after an extremely high dose. Otherwise, there were no side effects and no evidence of a thrombotic tendency. Conclusion: This recombinant concentrate is efficacious in FVII–deficient patients. It is safe since any risk of transmission of blood–borne viruses is eliminated.

This cross-sectional study included postmenopausal women, aged 45-75 years, with the aim to assess the presence of vulvovaginal atrophy (VVA) confirmed by a clinical assessment in the Italian population attending menopausal/gynecological centers. Apart from baseline variables, women scored vaginal, vulvar and urinary VVA symptoms. Impact of VVA on sexual function and quality of life (QoL) was assessed thorough EuroQoL questionnaire (EQ5D3L), Day-to-Day Impact of Vaginal Aging (DIVA), Female Sexual Function Index (FSFI) and Female Sexual Distress Scale-revised (FSDS-R). A physical examination was carried out in accordance with routine gynecological practice. VVA was confirmed in 90% of the 1226 evaluable patients (aged 59.0 ± 7.3 years). The prevalence of postmenopausal women with VVA confirmed by gynecological clinical assessment was 75.3%. The patients with VVA confirmed (n ¼ 926) had more severe symptoms (p < .0005), lower QoL (EQ-visual analog scale, p ¼ .008 and DIVA, p < .0005) and worsened sexual function (FSFI and FSDS-R, p < .0005 for both) when compared with the patients having nonconfirmed VVA (n ¼ 140). VVA is highly prevalent among postmenopausal Italian women. The objective of VVA confirmation is associated with severe symptoms and impaired QoL and sexual function. A proactive approach of Italian clinicians to promote regular and early gynecological evaluation should be performed in order to delay the advancing of the disorder.

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This prospective study was carried out with the aim of evaluating the efficacy of solvent/detergent inactivation of the hepatitis C virus (HCV) as applied to a chromatographic factor VIII concentrate. In parallel, the markers for other viruses, either lipid-enveloped (human immunodeficiency virus types 1 and 2 [HIV-1 and -2] and hepatitis B virus [HBV]) or non-lipid-enveloped viruses (such as B19 parvovirus and hepatitis A virus [HAV]) were evaluated. The study included 14 hemophilia centers, which enrolled 36 previously untreated patients (median age, 3 years; range, 1-56). The length of follow-up was 12 months, during which HCV (first- and second-generation assays and recombinant immunoblot assay), HIV-1 and -2, HBV, HAV (IgG and IgM), and parvovirus (IgG and IgM) antibodies, as well as alanine aminotransferase values were evaluated. Thirty-one patients were analyzable; none seroconverted for HCV, HBV, or HIV after exposure to a total of 165,000 IU of factor VIII (41 different lots). In one patient, alanine aminotransferase values rose to 167 mU per mL, 6 weeks after the first concentrate infusion, and this patient seroconverted for HAV 1 week later. Furthermore, 10 patients seroconverted for parvovirus during follow-up. This study suggests that the solvent/detergent method of virus inactivation is efficient in relation to lipid-enveloped blood-borne viruses but not in relation to non-lipid-enveloped viruses.

A study evaluating the risk of a commercial factor VIII (FVIII) concentrate's transmitting the human immunodeficiency virus (HIV) was carried out on hemophiliacs, by using multiple serological markers and the polymerase chain reaction (PCR). Twenty-nine hemophiliacs, negative for HIV antibodies, were treated for 18 months with a concentrate that had been inactivated by solvent-detergent. HIV-1 antibodies and antigen were assayed during the follow-up period. At the end of the study, all patients were also tested by the HIV 1 + 2 combined antibody assay; Western blot (WB) antibody analysis; and in eight cases, by an HIV-1 PCR technique. Patients received a yearly median FVIII dose of 35,330 IU (range 3,300-306,000); the median number of lots given to each patient was 6 (1-45). During the follow-up period and at the end of the study, HIV-1 antibodies and antigen were not detected in any of the subjects. The HIV 1 + 2 combined assay and WB analysis carried out only at the end of the study were negative. HIV-1 PCR was negative in all the tested patients. This study has shown that this solvent-detergent-treated FVIII concentrate did not transmit HIV.