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Welcome to I see Waldenström’s macroglobulinemia, a dedicated resource to help healthcare practitioners find the latest information about the signs, symptoms and diagnosis of WM.
Waldenström’s macroglobulinemia (WM) is a chronic, indolent, B-cell disorder characterised by bone marrow infiltration with lymphoplasmacytic cells, along with IgM monoclonal gammopathy.[1][2]
A quick overview of the mutation that leads to the development of WM, what are the disease manifestations, and the relevance of the asymptomatic disease.
CP-299789, approved on 11/05/2022
In Europe, the WM incidence rate is 7.3 per million in men, and 4.2 per million in women[3]
A strong familial predisposition has been reported,[3][5] first-degree relatives of WM patients have up to 20-fold increased risk for developing WM (and also increased risk but at lower level for other B-cell disorders).[3]
In contrast to multiple myeloma, WM prevalence is higher among Caucasians than other races.[3][6]
Have a look at the International Prognostic Scoring System for Waldenström's Macroglobulinemia (IPSSWM) to evaluate the probability of progression, and its correlation with median survival.
CP-299791, approved on 11/05/2022
The main risk factor for WM is the presence of IgM-monoclonal gammopathy of uncertain significance (MGUS).[7][8] Whilst in most people, MGUS remains stable and doesn’t cause problems or need treatment, there is a risk of MGUS developing into a cancer.[9] The presence of IgM-MGUS increases the risk of WM by 46 times vs. the general population.[10][11]
Other risk factors include:[10][11]:
WM cells are characterised by very specific immunophenotypes[3][^7]:
Positive for WM
IgM, CD19, CD20, CD22, CD25, CD27, CD79a
Variable for WM
CD5, CD10, CD23, CD138
Negative for WM
CD103
These characteristic immunophenotypes differentiate WM from other Non-Hodgkin lymphomas and from multiple myeloma (MM).[7]
Patients with WM present with different genetic variations, with the most common being del (6q) together with the somatic mutation in MYD88(L265P).[7] The latter has an important role in the growth and survival of WM cells.[1][7]
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A dedicated online resource for healthcare professionals to learn about the diagnosis and management of rare haematological malignancies: AL amyloidosis, Waldenström's macroglobulinemia, mantle cell lymphoma and acute myeloid leukaemia.
CD=cluster of differentiation; IgM=immunoglobulin M; MGUS=monoclonal gammopathy of uncertain significance; WM=Waldenström’s macroglobulinemia.
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