Acquired hemophilia

Abstract

Acquired hemophilia (AH) is an autoimmune hemostatic disorder mediated by autoantibodies directed against factor FVIII: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 aPTT 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.

Understanding recurrent pregnancy loss: recent advances on its etiology, clinical diagnosis, and management

“…Studies have shown that endocrine related pregnancy failures are likely to occur early in pregnancy, indicating that hormonal regulation might play crucial roles in attachment and early implantation of an embryo into the uterus [ 26 ]. The maternal hypercoagulable state of blood is a physiological change in the blood system during normal pregnancy [ 27 ], and when excessive hypercoagulability of the blood occurs, the blood develops a pathological condition which predisposes to thrombus formation called prethrombotic state (PTS). Many studies have demonstrated that PTS, either hereditary or acquired, is associated with an increased risk of RPL [ 28 , 29 ].…”

Manejo anestésico de paciente con Hemofilia tipo A. A propósito de un caso

“…podrían presentar dicha enfermedad en situaciones especiales como, por ejemplo, en el Síndrome de Turner o en situaciones de consanguinidad (heredando dos cromosomas X mutados, uno del padre hemofílico y el otro de la madre portadora) 1 .…”

“…La hemofilia, en términos generales, es un trastorno de la coagulación de origen genético, con un patrón hereditario recesivo asociado al cromosoma X. Es importante conocer que la tercera parte de las personas hemofílicas no tienen antecedentes familiares de este trastorno, tratándose de una mutación de novo 1 .…”

Cost-Effectiveness of rFVIIa versus pd-aPCC in the Management of Mild to Moderate Bleeds in Pediatric Patients with Hemophilia A with Inhibitors in Mexico

“…Of those, 72% are covered by the Instituto Mexicano del Seguro Social (IMSS). A further 32% of patients are covered by the Secretar ıa de Salud [52]. The experts (n=7) reported that around 60% of patients with hemophilia in Mexico are treated within IMSS, whereas 23% and 18% are treated within Seguro Popular and Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado (ISSSTE), respectively.…”