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Die chronische idiopathische thrombozytopenische Purpura

Chronic idiopathic thrombocytopenic purpura — Current therapeutic options, pathophysiology, clinical signs and diagnosis

Aktuelles Therapiekonzept und Einführung in Pathophysiologie, Klinik und Diagnostik

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Zusammenfassung

□ Pathophysiologie

Die chronische idiopathische thrombozytopenische Purpura (ITP) ist eine Autoimmunerkrankung, gekennzeichnet durch einen erhöhten Plättchenabbau, der durch die Bildung thrombozytenspezifischer Antikörper, meist der Klasse IgG, die sich gegen Oberflächenstrukturen der Thrombozyten richten, verursacht wird. Infolgedessen kommt es zu einer gesteigerten Elimination der Thrombozyten im retikuloendothelialen System. Während im Kindesalter die ITP in der Regel akut verläuft, spontan reversibel ist und meist keiner medikamentösen Therapie bedarf, ist die ITP im Erwachsenenalter eine relativ häufige chronische hämatologische Erkrankung.

□ Behandlung

Die Therapiemaßnahmen zielen auf die verminderte Bildung und Thrombozytenbindung der Autoantikörper sowie die Hemmung der gesteigerten Thrombozytenphagozytose durch das retikuloendotheliale System ab. Therapieziel ist die Anhebung der Thrombozyten über 100000/μl oder zumindest die Stabilisierung der Thrombozytenzahl ohne Blutungsneigung. Beim therapeutischen Vorgehen wird zwischen Notfall- und Langzeitherapie unterschieden.

Initial werden bei Nichtnotfällen Corticoide in einer Standarddosis (1 bis 2 mg/kg Körpergewicht Prednisonäquivalent) empfohlen, während bei therapierefraktärer chronischer ITP kürzlich über eine erfolgreiche hochdosierte Dexamethason-Stoßtherapie berichtet wurde. Besteht nach einer Splenektomie oder nach Anwendung von Immunglobulinen weiterhin kein Therapieerfolg, stehen zahlreiche alternative, zum Teil experimentelle Therapieverfahren zur Verfügung.

□ Schlußfolgerung

Die Behandlung der ITP im Erwachsenenalter besteht aus etablierten medikamentösen, immunologischen und operativen Maßnahmen. Ihre Anwendung in der Praxis wird abhängig gemacht vom Verlauf der Krankheit sowie drohenden oder eingetretenen Komplikationen. So können bis zu 75% der Patienten in Remission gebracht werden. Alternative Therapien bleiben refraktären Fällen vorbehalten.

Abstract

□ Pathophysiology

Chronic idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by platelet destruction due to an antiplatelet autoantibody, usually of the IgG class, which coats autologue platelets and leads to their elimination by the reticuloendothelial system (RES). While in childhood ITP is more usually an acute and self-limiting problem which needs no drug treatment, adult ITP is a relatively common chronic hematological disease.

□ Treatment

Treatment aimes at inhibition of antibody-production and binding on thrombocytes and thrombocyte phagocytosis by the RES. Therapy should result in a platelet count of >100,000/μl or at least in stabilization of the platelet count without bleeding. Therapeutic approaches were divided into emergency and long-term treatment. In patients who require non-emergency treatment conventional-dose corticoids (1 to 2 mg/kg/d prednisone) are recommended as initial treatment, whereas pulsed high-dose dexamethason is recently reported to be effective in refractory ITP. After unsuccessful splenectomy or if treatment with gammaglobulins fails alternative and partly experimental therapies may have to be used.

□ Conclusion

Treatment of adult ITP includes established medical, immunological and surgical measurements. Their application depends on diseases progression as well as imminent or manifest complications. Remission is achieved in up to 75% of all patients. Alternative treatments remain for refractory cases.

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Böcher, A., Hagmann, F.G. & Kreiter, H. Die chronische idiopathische thrombozytopenische Purpura. Med Klin 93, 707–718 (1998). https://doi.org/10.1007/BF03044807

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