RESUMEN
Inherited disorders of platelet function are a heterogeneous group. For optimal prevention and management of bleeding, classification and diagnosis of the underlying defect are highly recommended. An interdisciplinary guideline for a diagnostic approach has been published (AWMF # 086-003 S2K; Hämostaseologie 2014; 34: 201-212). Underlying platelet disorder, platelet count, age and clinical situation modify treatment. Exclusive transfusion of platelet concentrates may be inappropriate as potentially adverse effects can outweigh its benefit. A stepwise and individually adjusted approach for restitution and maintenance of haemostasis is recommended. Administration of antifibrinolytics is generally endorsed, but is of particular use in Quebec disease. Restricted to older children, desmopressin is favourable in storage pool disease and unclassified platelet disorders. Although licensed only for patients with Glanzmann thrombasthenia and alloantibodies, in clinical practice rFVIIa is widely used in inherited platelet disorders with severe bleeding tendency. This guideline aims at presenting the best available advice for the management of patients with inherited platelet function disorders.
Asunto(s)
Antiarrítmicos/uso terapéutico , Trastornos de las Plaquetas Sanguíneas/congénito , Trastornos de las Plaquetas Sanguíneas/terapia , Desamino Arginina Vasopresina/uso terapéutico , Factor VIIa/uso terapéutico , Hemorragia/terapia , Transfusión de Plaquetas/normas , Antiarrítmicos/normas , Trastornos de las Plaquetas Sanguíneas/diagnóstico , Niño , Preescolar , Femenino , Alemania , Hematología/normas , Hemorragia/congénito , Hemorragia/diagnóstico , Hemostáticos/uso terapéutico , Humanos , Lactante , Recién Nacido , Masculino , Pediatría/normas , Guías de Práctica Clínica como AsuntoAsunto(s)
Plaquetas/fisiología , Ciclooxigenasa 1/metabolismo , Hemorragia/diagnóstico , Heterocigoto , Complicaciones Posoperatorias/diagnóstico , Ácido 15-Hidroxi-11 alfa,9 alfa-(epoximetano)prosta-5,13-dienoico/química , Ácido 15-Hidroxi-11 alfa,9 alfa-(epoximetano)prosta-5,13-dienoico/farmacología , Adulto , Ácido Araquidónico/deficiencia , Células Cultivadas , Ciclooxigenasa 1/genética , Estudios de Asociación Genética , Hemorragia/etiología , Hemorragia/genética , Humanos , Masculino , Agregación Plaquetaria/efectos de los fármacos , Agregación Plaquetaria/genética , Pruebas de Función Plaquetaria , Polimorfismo de Nucleótido Simple , Complicaciones Posoperatorias/genética , Recurrencia , Tromboxanos/química , TranscriptomaRESUMEN
By means of continuous plasmafiltration, considerable and rapid improvement could be achieved with regard to the skin lesions as well as antibody titers of a patient suffering from pemphigus vulgaris. Apart from plasmafiltration, treatment with azathioprim and steroids proved to be effective. Neither significant side effects nor infectious complications have been observed.