RESUMEN
INTRODUCTION: Acquired haemophilia is a rare autoimmune disorder caused by autoantibodies directed in the majority of the cases against clotting factor VIII. This disorder is characterised by the sudden onset of bleeding that not rarely may be life-threatening and need transfusion support. Most reports on this condition describe the need for blood transfusions during the acute, haemorrhagic phase, but the number of transfused red cell units is often unknown. PATIENTS AND METHODS: In the last 5 years, 14 patients with acquired haemophilia A were identified in the transfusion and haemophilia centres of Verona and Castelfranco Veneto. The transfusion support for these 14 patients was analyzed in this retrospective survey. RESULTS: The 14 patients required a total of 183 red cell units. The average transfusion requirement was 13 red cells units/patient, with a range from 0 to 38 units. CONCLUSIONS: Eleven of the 14 patients studied needed strong transfusion support to enable any further management of the haemorrhages, as well as for eradication treatment of the autoantibodies to factor VIII. A relevant part of the management of haemorrhagic symptoms as well as the first choice for any further treatment (bleeding or the cure of the underlying disease) is transfusion of red blood cells.
Asunto(s)
Autoanticuerpos/sangre , Enfermedades Autoinmunes/sangre , Trastornos de la Coagulación Sanguínea/sangre , Transfusión de Eritrocitos , Factor VIII/inmunología , Hemorragia/terapia , Adulto , Anciano , Anciano de 80 o más Años , Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/tratamiento farmacológico , Trastornos de la Coagulación Sanguínea/tratamiento farmacológico , Trastornos de la Coagulación Sanguínea/etiología , Cuidados Críticos , Factor VIIa/uso terapéutico , Femenino , Hemorragia/etiología , Humanos , Masculino , Persona de Mediana Edad , Proteínas Recombinantes/uso terapéutico , Estudios RetrospectivosRESUMEN
We report the case of a 58-year-old woman with type 2B von Willebrand's disease who received regular infusions of the factor VIII/von Willebrand factor concentrate Haemate-P (2000 IU every other day) for 4 months to prevent recurrent bleeding from a malignant esophageal ulcer. Prophylaxis with Haemate-P was shown to be safe and effective in this patient, thus enabling completion of radiochemotherapy.
Asunto(s)
Neoplasias Esofágicas/complicaciones , Factor VIII/administración & dosificación , Hemorragia/etiología , Hemorragia/prevención & control , Enfermedades de von Willebrand/complicaciones , Factor de von Willebrand/administración & dosificación , Neoplasias Esofágicas/radioterapia , Femenino , Humanos , Persona de Mediana Edad , Enfermedades de von Willebrand/tratamiento farmacológicoRESUMEN
BACKGROUND: Cephalosporins are frequently associated with positive direct antiglobulin tests (DAT) and may rarely cause immune hemolytic anemia (IHA). We describe a patient who developed hemolytic anemia while she was receiving intravenous cefotetan. STUDY DESIGN AND METHODS: Immunohematologic studies of drug-dependent antibodies were performed by using cefotetan-treated red blood cells (RBCs) and untreated RBCs in the presence of cefotetan. RESULTS: The patient's serum contained antibodies that reacted with both drug-coated RBCs (adsorption mechanism) and with uncoated RBCs when cefotetan was added to the serum (immune complex mechanism). The prompt recognition of the problem and discontinuation of the drug prevented the onset of renal failure and rapidly resolved the hemolytic reaction. CONCLUSION: Our report underlines the importance of close laboratory and immunohematologic monitoring of patients treated with cephalosporins in order to recognize swiftly any hemolytic reaction due to these antibiotics thus reducing the chance of serious sequelae.