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Wiener klinische Wochenschrift

, Volume 118, Issue 9–10, pp 255–264 | Cite as

Autoimmunthrombozytopenie (AITP) des Erwachsenen: Klinik, Diagnose und Therapie

  • Klaus LechnerEmail author
  • Ansgar Weltermann
  • Ingrid Pabinger
Übersichtsarbeit

Zusammenfassung

Die Inzidenz der AITP ist 20–30/Million/Jahr. Die Diagnose kann gestellt werden, wenn eine isolierte Thrombozytopenie bei sonst normalem Blutbild und ohne Splenomegalie vorhanden ist. Zusätzliche Tests wie Knochenmarkuntersuchung, Bestimmung der Plättchenantikörper und/oder von Thrombopoetin sind nur in speziellen Fällen erforderlich. Bei einer Thrombozytenzahl unter 20.000/μl und/oder Blutungsneigung sollte mit einer Steroidtherapie, üblicherweise 1 mg/kg Prednisolon begonnen werden. Ca. 40 % der Patienten erreichen nach 3–6 Monaten eine komplette Remission. Patienten ohne Remission, die zur Erhaltung der Blutungsfreiheit mehr als 0,1 mg/kg/Tag Prednisolon benötigen, sollten splenektomiert werden. Das Risiko der (laparoskopischen) Splenektomie ist klein (0,2 % Mortalität, ca. 10 % nicht fatale Komplikationen, inklusive Milz/Portalvenenthrombosen). 80–90 % der Patienten erreichen postoperativ eine komplette oder partielle Remission, ein Viertel dieser Patienten rezidiviert jedoch wieder. Das Risiko einer tödlichen Pneumokokkensepsis bei präoperativer Impfung ist äußerst gering. Sehr blutungsgefährdet sind ältere Patienten mit Thrombozytenzahlen unter 20.000/μl trotz Splenektomie. Für diese Patienten sind die wirkungsvollsten Medikamente Cyclophosphamid, Azathioprin und Rituximab (Mabthera). Ingesamt ist das Risiko einer tödlichen Blutung bei der AITP jedoch gering und wahrscheinlich geringer als das Therapierrisiko bei aggressivem therapeutischem Vorgehen.

Schlüsselwörter

AITP Diagnostik Therapie Splenektomie Rituximab 

Adult autoimmune thrombocytopenia: diagnosis and treatment

Summary

The incidence of AITP is 20–30/million/year. The diagnosis is based on the finding of an isolated thrombocytopenia without other blood abnormalities and absence of a palpable spleen. Additional tests such as bone marrow examination, determination of platelet antibodies and of thrombopoetin are required only in special cases. The usual first line therapy in patients with bleeding tendency and a low platelet count is prednisolone at a dose of 1 mg/kg/day. Patients who have platelet counts of less than 20.000/μl 3–6 months after steroid therapy are candidates for splenectomy, in particular if more than 0.1 mg/kg/day prednisolone is required to keep the patient free of bleedings. Laparoscopic splenectomy has a low mortality (0.2 %) and morbidity (10 %). The risk of post-splenectomy overwhelming pneumococcal septicaemia can be minimized by preoperative vaccination. Older patients, who have low platelet counts after splenectomy, have a high bleeding risk. The most effective treatment options for these patients are cyclophosphamide, azathioprine and rituximab, but the choice of treatment should be carefully considered, since the risk of adverse effects may be greater than the risk of fatal bleeding.

Keywords

AITP Diagnosis Therapy Splenectomy Rituximab 

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Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Klaus Lechner
    • 1
    Email author
  • Ansgar Weltermann
    • 1
  • Ingrid Pabinger
    • 1
  1. 1.Abteilung Hämatologie/HämostaseologieUniversitätsklinik für Innere Medizin I, Medizinische Universität WienWienAustria

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