Waldenström macroglobulinaemia is a distinct B-cell lymphoproliferative disorder characterised by an immunoglobulin (Ig)M monoclonal gammopathy and bone marrow infiltration by lymphoplasmacytic lymphoma (LPL) (Owen et al, 2003). Clinical features are diverse and may relate to overall disease burden, such as peripheral blood cytopenias, organomegaly and constitutional symptoms, or may be directly attributable to the IgM paraprotein. The latter include hyperviscosity syndrome (HVS) and amyloidosis, as well as features attributable to autoantibody specificity, such as peripheral neuropathy, cold haemagglutinin disease (CHAD) and acquired von Willebrand disease (Treon, 2009).
These guidelines provide guidance for the management of patients of Waldenstrom's macroglobulinaemia (WM). The guideline makes recommendations on the investigations, diagnosis of WM and prognositic assessment.
The guideline makes recommendations on treatment, supportive care, assessment of response, monitoring and management of IgM related syndromes (hyperviscosity, neuropathy, cold haemagglutinin disease, cryoglobulinaemia and autoimmune cytopenias).