ABSTRACT
The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed for a median of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only 1 patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy. Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), whereas no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within 2 months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of nonsurvivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other nonsurvivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.
Subject(s)
Hemophilia A , Aged , Autoantibodies , Factor VIII , Female , Hemophilia A/diagnosis , Hemophilia A/drug therapy , Hemophilia A/epidemiology , Humans , Male , Registries , Retrospective StudiesABSTRACT
Los pacientes sometidos a trasplante de progenitores hematopoyéticos(TPH) reciben quimioterapia a altas dosis que les produce una pérdida de la inmunidad adquirida y con ello una situación de alto riesgo de infecciones. Para protegerse de ellas deben volver a cumplir un calendario de vacunas. Objetivo: conocer el grado de cumplimiento del calendario de vacunación, las causas que motivaron su incumplimiento y describir el papel que desempeña la enfermera. Material y método: estudio descriptivo transversal de las vacunas administradas durante los últimos 15 años a los 152 pacientes trasplantados en nuestro servicio. El instrumento de recogida de datos fue la hoja que se abrió a cada paciente al comienzo del calendario vacunal, diseñada conjuntamente por el equipo médico y enfermero, basado en la propuesta del Grupo Español de Trasplante Hematopoyético. Resultados: se completaron gran número de calendarios vacunales(63,8%), muchos de ellos sin incidencias (37,5%). La mayoría de las vacunas se administraron en la fecha prevista (91,2%) y un porcentaje elevado de incumplimientos fueron justificados (casi un 25% se debieron a fallecimientos, recaídas o progresiones). Se identificaron once actuaciones enfermeras diferentes relacionadas con el cumplimiento del calendario de vacunación. Conclusiones: los resultados obtenidos muestran que la actuación enfermera no se reduce tan solo a la administración y que la prescripción de vacunas tras el trasplante la realiza la enfermera, lo que constituye un ejemplo más de prescripción enfermera colaborativa (AU)
Patients undergoing hematopoietic stem cell transplantation (HSCT) receive high doses of chemotherapy makes them loose their acquired immunity and thus represents a high risk of infection. Order to protect them these patients should again follow an immunization schedule. Objective: To determine the degree of compliance with the immunization schedule, the reasons for noncompliance and to describe the role of the nurse. Material and methods: Cross-sectional descriptive study of the vaccines administered during the last 15 years to 152 patients transplanted in our department. The data collection instrument was data sheet that was opened for each patient at the beginning of the immunization schedule, designed jointly by the doctor and nurse team, based on the proposal of the Spanish Group for Hematopoietic Transplantation. Results: A large number of immunization schedules (63.8%), many without incidents, were completed (37.5%). Most vaccines were administered on the schedule date (91.2%) and a high percentage of failures were justified(almost 25% were due to exitus, relapses or progressions). We identified11 different nursing actions related to the implementation of the vaccination schedule. Conclusions: The results obtained show that the nursing performance is not reduced only to management and that prescribing vaccines after transplantation is performed by the nurse, which is another example of collaborative nurse prescribing (AU)
