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      Hemofilia adquirida Translated title: Acquired hemophilia

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          Abstract

          Resumen La hemofilia adquirida (HA) es un trastorno hemostático autoinmune ocasionado por autoanticuerpos dirigidos contra el factor VIII: C. En 52 % de los casos, la causa se desconoce o no se asocia con otra entidad patológica; en el resto, existen factores concomitantes: lupus, artritis reumatoide, cáncer, embarazo y medicamentos. En México no existe registro ni conciencia de la enfermedad entre el personal de salud. Los grupos de mayor incidencia son las mujeres en edad reproductiva y los individuos mayores de 70 años. Se caracteriza por hemorragia grave, sobre todo posterior a traumatismos y parto o cesárea, y equimosis grandes en tronco y extremidades. La sospecha es simple, basta que concurran hemorragia súbita, grave y un TTPa prolongado que no se corrige con plasma. El tratamiento consiste en lograr la hemostasia y erradicar el anticuerpo; lo primero se logra con el factor VII activado recombinante o concentrado del complejo de protrombínico activado. La ciclofosfamida, prednisona o rituximab sirven para erradicar el anticuerpo. La mayoría de los casos no son diagnosticados y la mortalidad es alta. Ya que los médicos desconocen el problema, no se sospecha, no se diagnostica y no se trata. Este documento revisa los datos más recientes de la HA y abunda en el diagnóstico y tratamiento.

          Translated abstract

          Abstract Acquired hemophilia (AH) is an autoimmune hemostatic disorder mediated by autoantibodies directed against factor VIII: C. In 52% of cases, the cause is unknown or is not associated with other pathological entities; in the rest, there are concomitant factors: lupus, rheumatoid arthritis, cancer, pregnancy, and medications. In Mexico, there is not a registry of AH, and awareness of the disease among health personnel is low. The groups with the highest incidence are women of childbearing age and individuals older than 70 years. It is characterized by severe bleeding, especially after trauma and normal childbirth or cesarean delivery, and large ecchymoses in the trunk and extremities. The suspicion is simple, it just takes for sudden, severe hemorrhage and a prolonged activated partial thromboplastin time that is not corrected with plasma to concur in an individual. Treatment involves achieving hemostasis and eradicating the antibody. The former is achieved with recombinant activated factor VII or activated prothrombin complex concentrate. Cyclophosphamide, prednisone or rituximab are used to eradicate the antibody. Most cases of AH are not diagnosed, which translates into a high mortality rate. Given that awareness about the disease among physicians is low, it is not suspected, neither diagnosed, and nor is it treated. This document reviews the most recent data on AH and expands on its diagnosis and treatment.

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          Author and article information

          Journal
          gmm
          Gaceta médica de México
          Gac. Méd. Méx
          Academia Nacional de Medicina de México A.C. (Ciudad de México, Ciudad de México, Mexico )
          0016-3813
          2696-1288
          February 2020
          : 156
          : 1
          : 67-77
          Affiliations
          [1] Ciudad de México orgnameInstituto Mexicano del Seguro Social orgdiv1Centro Médico Nacional La Raza orgdiv2Hospital de Especialidades “Dr. Antonio Fraga Mouret” Mexico
          [2] Ciudad de México orgnameInstituto Mexicano del Seguro Social orgdiv1Centro Médico Nacional La Raza orgdiv2Hospital de Especialidades “Dr. Antonio Fraga Mouret” Mexico
          Article
          S0016-38132020000100067 S0016-3813(20)15600100067
          10.24875/gmm.19005469
          32026881
          61d583a6-1e33-4c15-80a6-cb2249b4aa05

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

          History
          : 16 August 2019
          : 23 September 2019
          Page count
          Figures: 0, Tables: 0, Equations: 0, References: 49, Pages: 11
          Product

          SciELO Mexico

          Categories
          Artículos de revisión

          Autoimmunity,Autoanticuerpos,Autoinmunidad,Factor VIII,Hemofilia adquirida,Hemofilia,Autoantibodies,Acquired hemophilia,Hemophilia

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