JSH practical guidelines for hematological malignancies, 2018: II. Lymphoma-3. Lymphoplasmacytic lymphoma/Waldenström’s macroglobulinemia (LPL/WM)

  • Yukio KobayashiEmail author


In the 2017 WHO classification, lymphoplasmacytic lymphoma (LPL) is defined as a lymphoid neoplasm composed of a mixture of small B lymphocytes, lymphocytes with plasma cell differentiation, and plasma cells [1, 2]. It does not necessarily accompany IgM M-protein. Waldenström’s macroglobulinemia (WM) is defined as a subset of LPL accompanied by bone marrow involvement and IgM monoclonal gammopathy. Careful differential diagnosis is required as IgM monoclonal gammopathy is also a finding in other B cell lymphomas besides LPL.

Many patients (10–30%) present with hyperviscosity once the IgM M-protein concentration exceeds 3 g/dL. This can cause visual impairment (sausage-like changes of retinal veins in the fundus) and cerebrovascular disorders associated with agglutination of red blood cells. It can also be complicated by autoimmune disorders, cryoglobulinemia, peripheral neuropathy due to myelinolysis caused by antibody activity against myelin, amyloidosis, and coagulopathy or...

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© Japanese Society of Hematology 2019

Authors and Affiliations

  1. 1.Department of HematologyInternational University of Health and Welfare, Mita HospitalTokyoJapan

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