Lymphoplasmacytic lymphoma (LPL), sometimes called Waldenstrom's Macroglobulinemia, is an indolent (slow growing) lymphoma. It starts in plasma cells which develop from B lymphocytes. It is a rare disease representing only 1.5% of all NHLs diagnosed by lymph node biopsy in one study. Men are slightly more likely to get this lymphoma (53% vs. 47%) and the average patient age at diagnosis is 63 years old. The disease is more common among whites than blacks.

Symptoms: This lymphoma is usually associated with Waldenstrom's macroglobulinemia - a condition of increased monoclonal immunoglobulin paraprotien (IgM) greater than 3 grams per deciliter. Most patients have bone marrow, lymph node, and splenic involvement. Some patients may develop hyperviscosity syndrome. Typically when LPL is found, the patient is in later stages with lymph node, bone marrow, and spleen involvement. If it spreads outside the lymph system the lung and gastrointestinal tract are most likely to be involved.

Causes: The etiology (root cause) of LPL is unknown but it has been suggested that occupational exposure to paints, rubber dyes and leather may be a cause but this has not been confirmed. The disease has been reported in families suggesting a genetic link - gene translocation t(9;14)(p13;q32) and rearrangement of the PAX-5 gene are reported in some cases.. An association with hepatitis C was also reported.

Diagnosis & Staging: The following tests may be performed by your medical team during the diagnosis and staging of the disease: Full blood count, renal and liver function, serum uric acid, serum viscosity, cold agglutinins/cryoglobulins, beta two microglobulin, bone marrow aspiration and trephine biopsy, and CT scans.

Treatment of lymphoplasmacytic lymphoma is broken into two illnesses:

1) Treatment for hyperviscosity. This can be accomplished by plasmapheresis if treatment of the lymphoma is not reducing the IgM but it is not a cure.

2) Treating the lymphoma (see below)

Chemotherapy with chlorambicil has been used and is the treatment of choice in Europe with about a 70% response rate and a mean survival time of 5.4 years. CHOP chemotherapy has also been used with a response rate of 60% and median survival of 5 years. Fludarabine and 2-CDA have also been used.

High dose treatment with a stem cell transplant has been used in smaller numbers of patients - patient age should be considered when contemplating a stem cell transplant.

Rituxan has been tried in studies, especially with Waldenstrom's macroglobulinemia - it appears to decrease IgM but the results were rarely permanent, lasting a mean time of 8 months. Studies are being done combining Rituxan and fluarabine. Since Rituxan has an effect, other immunotherapies like bexxar may also be studied. Zevalin has entered into at least preliminary studies. Several studies have combined CHOP with rituxan for various lymphomas with positive results (abstract).

As in other lymphomas, the medical community should look to the latest studies and clinical trials to find advances in treatment. Patients may also look at resources such as PubMed for papers.

 

 

Resources

NCI Non-Hodgkin's Lymphoma Information
Information from Hematopathology
Information from a pathology update in the Cancer Control Journal: Indolent Lymphomas
NCI Cancer Facts paper on Waldenström’s Macroglobulinemia

Information Sources

The Hematopathology of Lymphoma - technical but full of very good information.
NCI Non-Hodgkin's Lymphoma Information
The Atlas of Genetics and Cytogenetics in Oncology and Haematology page on LPL

 

Related Articles

Adult Non-Hodgkin's Lymphoma Information Pages:

Non-Hodgkin's Lymphomas
Non-Hodgkin's Lymphoma: Diagnosis
Non-Hodgkin's Lymphoma: Aggressive Lymphomas
Non-Hodgkin's Lymphoma: Indolent Lymphomas
Non-Hodgkin's Lymphoma: Treatment
Non-Hodgkin's Lymphoma: Resources
Bone Marrow and Stem Cell Transplants

Other Pages of Interest:

Childhood Non-Hodgkin's Lymphoma Information
The Lymphoma Information Network

 

Books

Non-Hodgkin's Lymphomas, Peter M. Mauch (Editor), James O. Armitage (Editor), et al., 2004.

Section IV: Pathology, Biology, Clinical Evaluation, and Treatment Section

Chapter 15: Lymphoplasmacytic Lymphoma and Waldenstrom's Macroglobulinemia