Diagnosis and management of Waldenström macroglobulinaemia—A British Society for Haematology guideline

Abstract

Scope The objective of this guideline is to provide healthcare professionals with clear guidance on the management of patients with Waldenström macroglobulinaemia. In individual patients, circumstances may dictate an alternative approach. Methodology This guideline was compiled according to the British Society for Haematology (BSH) process at http://www.b-s-h.org.uk/guidelines/proposing-and-writing-a-new-bsh-guideline/. Recommendations are based on a review of the literature using Medline, Pubmed, Embase, Central, Web of Science searches from beginning of 2013 (since the publication of the previous guidelines) up to November 2021. The following search terms were used: Waldenström(’s) macroglobulin(a)emia OR lymphoplasmacytic lymphoma, IgM(‐related) neuropathy OR cold h(a)emagglutinin disease OR cold agglutinin disease OR cryoglobulin(a)emia AND (for group a only) cytogenetic OR molecular OR mutation OR MYD88 OR CXCR4, management OR treatment OR transfusion OR supportive care OR plasma exchange OR plasmapheresis OR chemotherapy OR bendamustine OR bortezomib OR ibrutinib OR fludarabine OR dexamethasone OR cyclophosphamide OR rituximab OR everolimus, bone marrow transplantation OR stem cell transplantation. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) nomenclature was used to evaluate levels of evidence and to assess the strength of recommendations. The GRADE criteria can be found at http://www.gradeworkinggroup.org. Review of the manuscript was performed by the British Society for Haematology (BSH) Guidelines Committee Haemato‐Oncology Task Force, the BSH Guidelines Committee and the Haemato‐Oncology sounding board of BSH. It was also on the members section of the BSH website for comment. It has also been reviewed by UK Charity WMUK; these organisations do not necessarily approve or endorse the contents.

Bing-Neel Syndrome: Real-Life Experience in Personalized Diagnostic Approach and Treatment

“…BNS transcends the simple definition of CNS involvement in WM. It can develop at any time point over the course of WM ( 15 ), regardless of whether the disease is active or amenable to therapy. As noted above, its symptoms may mirror those of other diseases and disorders (e.g., Parkinson’s disease, transient ischemic attack, seizures) resulting in significant delays in diagnosis and treatment.…”

Current Status of Novel Agents for the Treatment of B Cell Malignancies: What’s Coming Next?

“…While venetoclax exhibited marked single-agent activity in MCL and WM, its outcome in treating the other B neoplasms was not as satisfactory [ 3 , 60 , 66 ]. Many clinical trials have started to evaluate various combination regimens of venetoclax in NHL and B-ALL (reviewed in [ 3 , 30 , 59 , 60 , 61 , 63 ]). Two phase II/III trials are evaluating the efficacy of venetoclax + ibrutinib in patients with relapsed MCL (NCT03112174) [ 67 ] and in WM with a specific MYD88 gene mutation (NCT04273139).…”

Diagnostics in Waldenström’s macroglobulinemia: a consensus statement of the European Consortium for Waldenström’s Macroglobulinemia

“…Thus, patients with predominant lymphocytic infiltration and poor plasmacytic differentiation may have low serum IgM levels [8,33] and could be incorrectly classified as MGUS without a BM evaluation [34]. Consequently, although the value of BM assessment in asymptomatic individuals is not fully established, most groups currently agree that it may provide prognostic information about the risk of progression and the indication of the BM biopsy should be discussed [6].…”