Neue Entwicklungen beim Morbus Waldenström

 

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Zusammenfassung

Der Morbus Waldenström ist eine seltene Subentität in der Gruppe der indolenten B-Zell-Lymphome. Derzeitiger Standard in der Therapie ist eine Rituximab- / Chemotherapie. In letzter Zeit wurden bei Patienten mit einem Morbus Waldenström eine Reihe von genetischen Veränderungen entdeckt, von denen die MYD88 Mutation bei über 90 % der Patienten vorkommt und bei der Diagnose der Erkrankung helfen kann. Mit der Einführung des oralen Bruton-Tyrosinkinaseinhibitor Ibrutinib steht eine effektive chemotherapiefreie Behandlung zur Verfügung.

Abstract

Waldenström’s Macroglobulinemia (WM) is a rare subtype of B-cell lymphoma with mostly indolent course. Until now the backbone of therapy is still Rituximab / Chemotherapy. In recent years whole genome sequencing has revealed a number of genetic mutations, among them mutations of the MYD88 gene which occurs in over 90 % of patients and helps to validate diagnosis of WM in difficult cases. With the introduction of ibrutinib, an oral bruton-tyrosine-kinase inhibitor, an effective chemotherapy-free treatment option is available.

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