Waldenström's macroglobulinemia/lymphoplasmacytic lymphoma

Abstract

Waldenström's macroglobulinemia (WM) is included in the World Health Organization classi fication as the lymphoplasmacytic lymphoma. It is a rare type of non-Hodgkin lymphoma (NHL) with distinct clinicopathological features resulting from the accumulation of clonally related B lymphocytes, lymphoplasmacytic cells, and plasma cells which secrete a monoclonal IgM protein. Unlike other types of NHL, WM is rarely associated with lymphadenopathy or splenomegaly. WM has a chronic clinical course and treatment options are usually different from other types of indolent B-cell lymphoma. In this chapter, we will review the most recent data on the biology of WM and current treatment strategies.