DRZ

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.