Guidelines on the diagnosis and management of Waldenstrom macroglobulinaemia-A British Society for Haematology guideline

Scope The objective of this guideline is to provide healthcare professionals with clear guidance on the management of patients with Waldenstrom 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 . 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: Waldenstrom('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 . 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.

Automated summary

This guideline aims to provide healthcare professionals with clear guidance on the management of patients with Waldenstrom macroglobulinaemia. It is based on a comprehensive review of the literature and provides recommendations on management, treatment, and supportive care. The guideline was developed and reviewed following a rigorous methodology.