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Chronische lymphatische Leukämie

Therapie und genetisches Risikoprofil

Chronic lymphocytic leukemia

Treatment and genetic risk profile

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Zusammenfassung

Die chronische lymphatische Leukämie (CLL) zeigt einen extrem variablen klinischen Verlauf. Diese Heterogenität ist begründet durch genetische Faktoren wie genomische Aberrationen und den Mutationsstatus der Immunglobulingene (IGHV). Die Therapieoptionen bei der CLL wurden über die vergangenen Jahre erheblich erweitert. Die Kombination aus Fludarabin, Cyclophosphamid und Rituximab (FCR) wurde als wirksamste Standardtherapie bei körperlich fitten Patienten etabliert. Bendamustin kombiniert mit Rituximab (BR) wird gegenwärtig in Studien mit FCR verglichen. Chlorambucil ist weiterhin relevant bei älteren Patienten mit Komorbidität. Alemtuzumab ist eine Alternative für Hochrisikopatienten (refraktäre CLL, 17p-Deletion, TP53-Mutation). Als einzige kurative Option bietet sich die allogene Stammzelltransplantation an, allerdings verbunden mit einer erheblichen Mortalität. Innovative Therapieansätze konzentrieren sich auf eine gezielte sowie individualisierte Therapie und zeigen nach ersten Daten erhebliche Wirksamkeit bei hervorragender Verträglichkeit. Diese Übersichtsarbeit beschreibt die derzeitigen Standardtherapien und vielversprechende neue Behandlungsansätze.

Abstract

Chronic lymphocytic leukemia (CLL) is characterized by a highly variable clinical course. Among the biological features underlying this heterogeneity, genetic lesions and the mutational status of the immunoglobulin heavy chain variable genes (IGHV) are of importance. Therapeutic options in CLL have been considerably expanded during recent years. The combination of fludarabine, cyclophosphamide and rituximab (FCR) has become gold standard in the first-line treatment of physically fit patients. Bendamustine plus rituximab (BR) is currently being compared to FCR in studies and chlorambucil is still of relevance for elderly patients with comorbidities. Alemtuzumab is an alternative for high-risk patients (refractory CLL, 17p deletion, TP53 mutation). Allogeneic stem cell transplantation (allo-SCT) offers the only chance of cure but not without substantial mortality. Innovative approaches focus on individualized, targeted therapies. A number of novel agents are in clinical trials and show marked efficacy combined with good tolerability. This review provides an overview of the current therapeutic options and of promising novel approaches.

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Interessenkonflikt

Der korrespondierende Autor weist für sich und seinen Koautor auf folgende Beziehungen hin: Drittmittel und Honorare von: Roche, Genzyme, Gilead, Amgen, GSK, Mundipharma, Celgene.

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Authors and Affiliations

  1. Klinik für Innere Medizin III, Universitätsklinikum Ulm, Albert-Einstein-Allee 23, 89081, Ulm, Deutschland

    S. Stilgenbauer

  2. Klinik für Innere Medizin I, Universitätsklinikum Köln, Köln, Deutschland

    M. Hallek

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  1. S. Stilgenbauer
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  2. M. Hallek
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Correspondence to S. Stilgenbauer.

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Stilgenbauer, S., Hallek, M. Chronische lymphatische Leukämie. Internist 54, 164–170 (2013). https://doi.org/10.1007/s00108-012-3153-z

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