2
views
0
recommends
+1 Recommend
1 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Rituximab en combinación con agentes "bypass" hemostáticos para hemofilia adquirida con inhibidor, a propósito de un caso Translated title: Rituximab in combination with hemostatic "bypass" agents for hemophilia acquired with an inhibitor, in the event of a case

      case-report

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Resumen La hemofilia adquirida es un trastorno hemorrágico producido por anticuerpos del propio organismo que actúan frente al factor VIII. Posee una incidencia estimada 1-1,5 casos/millón/año, con predominio en sujetos de edad avanzada. Es considerada una enfermedad rara pero su impacto puede verse infraestimado por la falta de registros adecuados, la falta de conocimiento por el facultativo y su complejidad para diagnosticarla. No hay establecido un protocolo fijo en el manejo de la patología, por ello la mayoría de las recomendaciones se basan en las opiniones de expertos en el tratamiento de otras coagulopatías.

          Translated abstract

          Abstract Acquired hemophilia is a bleeding disorder caused by the body's own antibodies to factor VIII. It has an estimated incidence of 1-1.5 cases/million/year, with a predominance of elderly subjects. It is considered a rare disease but its impact may be underestimated due to the lack of adequate records, the lack of knowledge by the specialists and its complexity in diagnosing it. There is no fixed protocol in the management of the pathology, so the majority of recommendations are based on the opinions of experts in the treatment of other coagulopathies.

          Related collections

          Most cited references5

          • Record: found
          • Abstract: found
          • Article: not found

          Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry.

          Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%).
            Bookmark
            • Record: found
            • Abstract: not found
            • Article: not found

            Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management.

              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Acquired factor VIII inhibitors.

              Acquired hemophilia A is a rare bleeding diathesis caused by autoantibodies directed against clotting factor VIII and associated with an increased morbidity and mortality. This autoimmune disorder most commonly occurs in the elderly. Although it may be associated with several underlying pathologies, up to 50% of reported cases remain idiopathic. In contrast with congenital hemophilia, which is commonly characterized by hemarthroses, hemorrhages in patients with acquired hemophilia involve most frequently soft tissues. The 2 treatment priorities are to arrest the acute bleeding and to eradicate the factor VIII autoantibody. Acute bleeding episodes in patients with low-titer inhibitors can be treated using human factor VIII concentrates, whereas factor VIII bypassing agents, such as activated prothrombin complex concentrates or recombinant activated factor VII, are effective for the treatment of those with high-titer inhibitors. An analysis of the literature shows that the most effective first-line treatment for the eradication of factor VIII autoantibodies is the combination of steroids and cyclophosphamide. However, there is increasing evidence on the effectiveness of other treatment approaches, such as immune tolerance regimens and rituximab. If confirmed by large controlled studies, these innovative therapies might become a valid option for long-term eradication of factor VIII inhibitors.
                Bookmark

                Author and article information

                Journal
                ofil
                Revista de la OFIL
                Rev. OFIL·ILAPHAR
                Organización de Farmacéuticos Ibero-Latinoamericanos (Madrid, Madrid, Spain )
                1131-9429
                1699-714X
                December 2021
                : 31
                : 4
                : 433-434
                Affiliations
                [2] Almería orgnameHospital Universitario Torrecárdenas orgdiv1Servicio de Hematología España
                [1] Almería orgnameHospital Universitario Torrecárdenas orgdiv1Servicio de Farmacia España
                Article
                S1699-714X2021000400020 S1699-714X(21)03100400020
                10.4321/s1699-714x20210004000020
                04dcaafb-a517-4da6-8218-765b7e1ed7a2

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 18 August 2020
                : 14 July 2020
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 5, Pages: 2
                Product

                SciELO Spain

                Categories
                Casos Clínicos

                rituximab,anticuerpos,factores inmunológicos/uso terapéutico,factor VIII/antagonistas e inhibidores,Hemophilia A/blood,antibodies,immunologic factors/therapeutic use,factor VIII/antagonists & inhibitors,Hemofilia A/sangre

                Comments

                Comment on this article