Current options to manage Waldenström’s macroglobulinemia

ABSTRACT

Introduction: Waldenström’s macroglobulinemia (WM) is a rare, incurable B-cell lymphoma, with a median survival of 5–10 years in symptomatic patients. There is no consensus on the standard of care and several agents are currently used in these patients.

Areas covered: In this article, we will review the use of standard therapies and new drugs investigated such as monoclonal antibodies, proteasome inhibitors, immunomodulatory agents, Bruton’s tyrosine kinase inhibitors and novel agents in early-stage development.

Expert commentary: RCD (Rituximab/Cyclophosphamide/Dexamethasone) is an effective and safe treatment in first line in WM. BR (Bendamustine/Rituximab) or BRD (Bortezomib/Rituximab/Dexamethasone) provide durable responses, and are still indicated in most patients. Ibrutinib is a new option and it was approved as primary therapy and for relapse. Carfilzomib based therapy represents an emerging option for proteasome-inhibitor based therapy for WM. Despite encouraging results, WM remains an incurable disease; therefore, new treatment options are needed. For this reason, continued participation in clinical trials should be encouraged.

KEYWORDS:

• Waldenström’s macroglobulinemia
• new drugs
• treatment options
• monoclonal antibody
• Bruton’s tyrosine kinase inhibitors

Declaration of interest

G Benevolo is on the advisory board for Janssen, Novartis and receives lecture fees from Janssen, Novartis and Bristol-Myers Squibb. V Umberto is on the advisory board for Roche, Janssen, Celgene and receives lecture fees from Roche, Janssen and Celgene. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Additional information

Funding

The manuscript was not funded.