BSH 2022 | An update on the BSH guidelines for the diagnosis and management of Waldenström’s macroglobulinemia

So, we have recently published the latest update on the BSH guidelines on the diagnosis and management of WM. And they’re really in three sections, there’s firstly, the diagnosis and investigations of patients with WM, the management, and then talking about some of the complications that occur with WM. From the diagnosis side of things, the real thing that has been updated compared to the previous guidelines is the use of genomics in helping us understand and define WM as opposed to other indolent B-cell lymphomas. So, obviously MYD88 Mutation has had a big impact in us being able to diagnose these patients. Also, from the diagnosis and investigation side, we’ve tried to make these guidelines quite practical for clinicians. So, to think about the different mechanisms with which people can get some of the complications. So for example, if a patient of yours with newly diagnosed WM has a bleeding tendency, they’re not just to think about checking a clotting screen, but also to think about acquired Von Willebrands, could this patient have amyloid, and to think about some of the complications that could all lead to the same symptomatology.

From the treatment side of things, we’ve gone through in detail the trial data showing the evidence for the different treatment options that are available. And as with all BSH guidelines, we haven’t limited the treatment analysis and our recommendations to what is currently available and funded in the UK, but based it on published evidence. And again, the real paradigm shift compared to the previous guidelines is the advent of the era of targeted therapies and the big change that having the availability of BTK inhibitors have had in terms of therapy options for our patients. And so, we’ve gone through the evidence for the different BTK inhibitors available.

And then the last section is about the complications and managing the holistic care of the patient with WM. And again, I think there’s too many complications that can occur with WM, partly related to the lymphoma aspect of it, partly related to the paraprotein and some of the unique properties of the paraprotein, but there are general advice that we’ve given and recommendations that potentially would be involving other teams where necessary. So for example, if a patient has amyloid, getting the amyloid center involved. If the patient has neurological symptoms or problems involving our neurology colleagues at both in terms of investigations, but also in terms of management and we couldn’t talk about all the complications in detail in the guidelines, so it’s signposting to some of the other guidelines that could be relevant in this scenario.