Lymphoplasmacytic Lymphoma

At a Glance

Lymphoplasmacytic lymphoma is a low grade small B-cell non-Hodgkin lymphoma that has a monoclonal B-cell and a monoclonal plasma cell component. Most patients with lymphoplasmacytic lymphoma have Waldenström macroglobulinemia, which is lymphoplasmacytic lymphoma involving the bone marrow and IgM monoclonal gammopathy. It occurs in adults with a median age of 60 at diagnosis. It is slightly more common in men.


Lymphoplasmacytic lymphoma is diagnosed by a combination of clinical and pathological findings. Patients may present with a variety of clinical signs and symptoms, but some common laboratory findings are anemia, an IgM paraprotein (usually IgM kappa), hyperviscosity, cryoglobulinemia, and coagulopathy. Bone marrow biopsy is the most common way to diagnose lymphoplasmacytic lymphoma.

Flow cytometry studies are very helpful to identify the monoclonal B-cell and plasma cell populations. Cytogenetic studies are usually performed, but there is no specific chromosomal or oncogenic abnormality that characterizes lymphoplasmacytic lymphoma. No specific fluorescence in situ hybridization (FISH) testing is done, unless there is a concern for another type of lymphoma with a specific chromosomal translocation. Molecular polymerase chain reaction (PCR) studies to look for IgH gene rearrangements are not routinely done.


Sometimes, distinguishing between lymphoplasmacytic lymphoma and other small B-cell lymphomas, such as marginal zone lymphoma, is difficult. A patient may receive a diagnosis of "small B-cell lymphoma with plasmacytic differentiation" when this is the case. Waldenström macroglobulinemia can run in families. Hepatitis C viral infection is associated with lymphoplasmacytic lymphoma and type II cryoglobulinemia. Patients with lymphoplasmacytic lymphoma with Waldenstrom macroglobulinemia can develop autoimmune phenomena, hyperviscosity, cryoglobulinemia, coagulopathy, skin abnormalities, and diarrhea thought to be due to the IgM paraprotein. Sometimes, the spleen, lymph nodes, liver, and peripheral blood are involved by lymphoplasmacytic lymphoma.


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