Waldenström’s macroglobulinemia (WM) is a chronic, indolent, B-cell disorder characterised by bone marrow infiltration with lymphoplasmacytic cells, along with IgM monoclonal gammopathy.
In Europe, the WM incidence rate is 7.3 per million in men, and 4.2 per million in women
A strong familial predisposition has been reported, 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).
In contrast to multiple myeloma, WM prevalence is higher among Caucasians than other races.
WM cells are characterised by very specific immunophenotypes[^7]:
Positive for WM
IgM, CD19, CD20, CD22, CD25, CD27, CD79a
Variable for WM
CD5, CD10, CD23, CD138
Negative for WM
These characteristic immunophenotypes differentiate WM from other Non-Hodgkin lymphomas and from multiple myeloma (MM).
Patients with WM present with different genetic variations, with the most common being del (6q) together with the somatic mutation in MYD88(L265P). The latter has an important role in the growth and survival of WM cells.
CD=cluster of differentiation; IgM=immunoglobulin M; MGUS=monoclonal gammopathy of uncertain significance; WM=Waldenström’s macroglobulinemia.
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ITEM CODE: CP-231043 | DATE OF PREPARATION: June 2021.