Physician Info

The IgM-related peripheral neuropathies (IgM-PN) are a group of chronic disorders characterized by the presence of monoclonal IgM that may be associated with one of several diseases affecting the peripheral nerves. Neurological symptoms in this setting can also result from direct invasion of the peripheral or central nervous system by lymphoplasmacytic cells (neurolymphomatosis and Bing-Neel syndrome respectively) or via other mechanisms (for example AL amyloid deposition or cryoglobulinaemic vasculitis). A panel of experts discussed common clinical problems in the diagnosis, evaluation and treatment of patients with IgM-PN.  The consensus of the discussions is presented here.

Consensus panel 2 from the 12th International Workshop on Waldenstrom Macroglobulinemia was tasked with updating the guidelines on the diagnosis and management of patients with Bing-Neel syndrome (BNS).  The report, available here, summarizes the clinical symptoms that may be present with BNS, discusses the criteria required for diagnosis of BNS, makes recommendations for follow-up imaging, and proposes revised guidelines for response assessment in BNS.

Histological transformation (HT) in Waldenström's macroglobulinemia (WM) is a rare complication and despite growing literature in the last years, no consensus recommendations exist.  This panel was convened to review the current data on transformed WM and make recommendations on its diagnosis and management.  The report can be read here.

Consensus Panel 5 aimed to establish working definitions for intolerance and resistance to covalent BTK inhibitors, as well as provide strategies to identify and manage these issues not infrequently encountered in clinical practice.  The report can be read here.

Approximately 95% of lymphoplasmacytic lymphomas (LPL) are IgM secreting and are characterized as Waldenstrom Macroglobulinemia (WM). Conversely, non-IgM secreting LPL are rare. As part of the 12th International Workshop on WM (IWWM-12), a consensus panel of experts was tasked to develop recommendations for the management and response assessment of non-IgM LPL.  Their report is available here.

The Consensus Panel 3 (CP3) of the 12th International Workshop on Waldenström macroglobulinemia (IWWM-12) has reviewed and incorporated current data to make recommendations for the management of patients with high-risk WM (HR-WM).  The report can be read here.

Clinical trial NCT05006716 of Beigene’s BGB-16673, a Bruton Tyrosine Kinase Targeted Protein Degrader, is recruiting patients in Calgary, Edmonton, Québec, Vancouver, and Toronto.    Official title of the study: A Phase 1/2, Open-Label, Dose Escalation and Expansion Study of the Bruton Tyrosine Kinases Targeted Protein Degrader BGB-16673 in Patients with B-cell Malignancies.  For more information, see the trial's description on the NIH website.

Two journal article provide an end-to-end review of WM.  Co-authored by experts from the Dana-Farber Cancer Institute, they are highly recommended to all practitioners. Part 1: Epidemiology, Pathogenesis, Clinicopathologic Characteristics, Differential Diagnosis, Risk Stratification, and Clinical Problems is available at https://www.mjhid.org/mjhid/article/view/5732/4535 Part 2: Focus on Therapy is available at https://www.mjhid.org/mjhid/article/view/5878/4639  

NCT05952037 (https://clinicaltrials.gov/study/NCT05952037) is using a new BCL-2 inhibitor, Sonrotoclax, and is strictly for WM patients.  The trial is world-wide, but is recruiting in just one location in Canada, in North Vancouver, at the Lions Gate Hospital Chemotherapy Clinic.  It has several arms running in parallel:  one is for treatment-naive patients; the other three are for Relapsed/Refractory patients with various treatment histories.

This booklet provides a compilation of current knowledge on the diagnosis, treatment, and established protocols for WM.  Complete with references to published papers, throughout, it has been modified with a small number of Canadian-specific footnotes.  Authored by two WM specialist MDs from the Bing Center for WM at the Dana-Farber Cancer Institute, it is presented in partnership with the International Waldenstrom's Macroglobulinemia Foundation (IWMF).