Tumours of haematopoietic and lymphoid tissues
Lymphoplasmacytic lymphoma (LPL) is a neoplasm of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow (BM) and sometimes lymph nodes and spleen, which does not fulfill the criteria for any of the other small B-cell lymphoid neoplasms that may also have plasmacytic differentiation. Because the distinction between LPL and one of these other lymphomas, especially some marginal zone lymphomas (MZL), is not always clear-cut, some cases may need to be diagnosed as a small B-cell lymphoma with plasmacytic differentiation and a differential diagnosis provided. Although often associated with a paraprotein usually of IgM type, it is not required for the diagnosis. Waldenström macroglobulinemia
(WM) is found in a significant subset of patients with LPL and is defined as LPL with BM involvement and an IgM monoclonal gammopathy of any concentration
Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML, Morra E, Pangalis GA, San Miguel JF, Branagan AR, Dimopoulos MA (2003)
Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia.
Semin Oncol 30: 110-5
> Variant: Gamma heavy chain disease
Gamma heavy chain disease results from the secretion of a truncated gamma chain, which lacks light-chain binding sites. It is usually associated with a lymphoma that fulfills the criteria for LPL involving lymph nodes, BM, liver, spleen and PB but some cases resemble plasma cell myeloma. The clinical course is variable but probably more aggressive than that of typical IgM-producing LPL.
> Related Topics
Introduction: Classification of lymphoid neoplasms