Thrombotisch-thrombozytopenische Purpura (TTP) – Fallbericht, Pathogenese und Therapieoptionen

Zusammenfassung

Die thrombotisch-thrombozytopenische Purpura ist eine im Kindesalter sehr seltene Erkrankung. Die Prognose der Erkrankung ist wesentlich von dem raschen Beginn einer adäquaten Therapie abhängig. Als wichtigstes Element einer Therapie wird aktuell die Durchführung einer Plasmapharese gesehen. Die weiteren Therapieempfehlungen differieren zum Teil erheblich. Hier wird der Fall eines bei Erstdiagnose 12 Jahre alten Jungen präsentiert, der an zwei Episoden einer typischen idiopathischen TTP erkrankte. Während dieser Krankheitsepisoden konnten bei völligem Fehlen der Metalloprotease ADAMTS13 Antikörper gegen dieselbe nachgewiesen werden. Beide Krankheitsepisoden wurden mit täglicher Plasmapharese und Gabe von Glukokortikoiden therapiert. Bei ausbleibender Besserung wurde während der zweiten Episode zusätzlich Cyclophosphamid und später Rituximab gegeben. Eine anhaltende Remission konnte in beiden Fällen jedoch erst nach Therapie mit Vincristin erreicht werden.

Summary

The thrombotic-thrombocytopenic purpura (TTP) is a rare disease in childhood. The prognosis of the disease essentially depends on an immediate induction of the adequate therapy. Plasmapheresis is regarded as the most important therapy option, other therapeutic options vary. We report about a boy with two episodes of typical idiopathic TTP who was first presented at an age of 12 years. During these episodes antibodies against the metalloprotease ADAMTS13 were detected while the specific metalloprotease itself was absent. Plasmapheresis was daily instituted and glucocorticoids were administered. Additionally, cyclophosphamid and later rituximab was given because of lack of improvement during the second episode. However, persistent remission was achieved only after therapy with vincristin.

Schlüsselwörter

Thrombotisch-thrombozytopenische Purpura im Kindesalter - Metalloprotease - Cyclophosphamid - Vincristin - Rituximab

Keywords

Thrombotic thrombocytopenic purpura - metalloprotease - cyclophosphamid - vincristin - rituximab

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