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Current options to manage Waldenström's macroglobulinemia
Giulia Benevolo1, Maura Nicolosi1, Elisa Santambrogio1
1a Division of Hematology , AOU Città della Salute e della Scienza , Torino , Italy.
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.
Insights
Waldenström's macroglobulinemia (WM) treatment involves standard therapies like RCD and BRD, alongside new drugs such as ibrutinib. While effective, WM remains incurable, necessitating ongoing clinical trial participation.
Area of Science:
- Hematology
- Oncology
- Pharmacology
Background:
- Waldenström's macroglobulinemia (WM) is a rare, incurable B-cell lymphoma.
- Current standard of care lacks consensus, with various agents used.
- Median survival for symptomatic patients is 5-10 years.
Purpose of the Study:
- To review standard therapies for WM.
- To discuss novel therapeutic agents under investigation for WM.
- To provide expert commentary on current and emerging WM treatments.
Main Methods:
- Review of existing literature on WM therapies.
- Analysis of clinical data for standard and investigational drugs.
- Expert opinion on treatment efficacy and safety.
Main Results:
- Rituximab/Cyclophosphamide/Dexamethasone (RCD) is effective and safe for first-line WM treatment.
- Bendamustine/Rituximab (BR) or Bortezomib/Rituximab/Dexamethasone (BRD) offer durable responses.
- Ibrutinib is approved for primary and relapsed therapy; Carfilzomib-based therapy is an emerging option.
Conclusions:
- WM remains an incurable disease despite encouraging treatment results.
- New therapeutic options are continuously needed.
- Encouraging patient participation in clinical trials is crucial for advancing WM treatment.
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