"Waldenstrom's Macroglobulinemia is a rare, chronic cancer that is classified as a plasma cell neoplasm. It affects plasma cells, which develop from white blood cells called B-lymphocytes, or B cells.
B cells form in the lymph nodes and the bone marrow, the soft, spongy tissue inside bones. They are an important part of the body's immune (defense) system. Some B cells become plasma cells, which make, store, and release antibodies. Antibodies help the body fight viruses, bacteria, and other foreign substances.
In Waldenstrom's Macroglobulinemia, abnormal plasma cells multiply out of control. They invade the bone marrow, lymph nodes, and spleen and produce excessive amounts of an antibody called IgM. Excess IgM in the blood causes hyperviscosity (thickening) of the blood.
Waldenstrom's Macroglobulinemia usually occurs in people over age 65, but can occur in younger people. A review of cancer registries in the United States found that the disease is more common among men than women and among whites than blacks.
Some patients do not experience symptoms. Others may have enlarged lymph nodes or spleen, and may experience fatigue, headaches, weight loss, a tendency to bleed easily, visual problems, confusion, dizziness, and loss of co-ordination. These symptoms are often due to the thickening of the blood. In extreme cases, the increased concentration of IgM in the blood can lead to heart failure.
The diagnosis of Waldenstrom's Macroglobulinemia generally depends on the results of blood and urine tests and a bone marrow biopsy. During this test, a needle is inserted into a bone and a small amount of bone marrow is withdrawn and examined under the microscope.
The initial treatment of Waldenstrom's Macroglobulinemia is determined mainly by the thickness of the patient's blood. Patients with hyperviscosity usually receive chemotherapy (anticancer drugs). A type of treatment called plasmapheresis may be performed to relieve symptoms such as excessive bleeding and dizziness. In this procedure, blood is removed from the patient and circulated through a machine that separates the plasma (which contains the antibody IgM) from the other parts of the blood (red blood cells, white blood cells, and platelets). The red and white blood cells and platelets are returned to the patient, along with a plasma substitute. Interferon alpha, a biological therapy (a type of treatment that stimulates the immune system to fight cancer) can also help to relieve symptoms. For long-term control of the disease, doctors generally combine plasmapheresis with chemotherapy."
"Lymphoplasmacytic-lymphoplasmacytoid lymphoma (LPL) / Waldenstrom's macroglobulinemia (WM) or immunocytoma (IMC) consists of diffuse proliferation of small mature B lymphocytes, plasmacytoid lymphocytes, and plasma-cells. The nosographic definition includes the lack of histological, immunophenotypic, cytogenetic, and molecular markers considered specific of other types of lymphoma. The cells show surface Ig (usually IgM), B-cell-associated antigens and display the CD5-, CD23- and CD10- phenotype, which allows for differential diagnosis from B-CLL and mantle cell lymphoma. t(9;14)(p13;q32) chromosomal translocation has been found in 50% of all LPL cases. The cytogenetic rearrangement juxtaposes the PAX-5 gene, which encodes for an essential transcription factor for B-cell proliferation and differention, to the Ig heavy chain gene.
The combination of chlorambucil and prednisone holds as the standard treatment and seems to guarantee good control of the disease in most patients. Similar therapeutic results have been described with the combination of cyclophosphamide, vincristine, prednisone with (CHOP) or without doxorubicin (CVP), or with a combination of other alkylating agents and prednisone. Nucleoside analogues, alone or in combination with alkylating agents and anthracyclines, provide good salvage therapy for IMC and being increasingly employed as first line therapy.
In a multicentric European trial Foran et al. administered the chimeric anti-CD20-monoclonal antibody (Rituximab) to 28 patients with previously treated IMC. Seven out of 25 evaluable patients (28%) achieved a partial response. Byrd et al. examined the outcome of 7 previously treated WM patients who received weekly infusions of rituximab (375 mg/m2). Therapy was well tolerated by all patients, and there was no decrease in cellular immune function, or significant infectious morbidity. Partial responses were noted in three of these patients, including two with fludarabine-refractory disease. These data suggest that rituximab exerts clinical activity on heavily pre-treated patients with WM. Furthermore, Weide et al. first reported that WM-associated polyneuropathy can be treated effectively with a combination of chemotherapy and the anti-CD20 monoclonal antibody rituximab. Most published trials exploring the efficacy of high dose treatment as salvage therapy for relapsed or refractory low grade non Hodgkin's lymphoma have included prevalently follicular or lymphocytic lymphomas. In selected high risk patients radioimmunotherapy with autologous stem-cell rescue, and myeloablative therapy followed either by autologous stem cell transplantation (SCT) or allogeneic SCT might represent an alternative strategy."
To subscribe to the discussion and support list for Waldenstrom's macroglobulinemia, contact the International Waldenstrom's Macroglobulinemia Foundation at www.iwmf.com.
If you would like to follow the announcements regarding the latest research for low grade lymphomas, Ben Haines' list is the place to be.
To subscribe: send an empty message to nhllow-subscribe@egroups.com. Or you can follow the messages from the archives: go to egroups.com and do a search, then enter the nhllow list.
To unsubscribe: send an empty message to nhllow-unsubscribe@egroups.com.
For those interested in alternatives and complementary treatments, there is a NHL list for discussing them and sharing experiences. To subscribe, send an empty message to nhl-info-subscribe@egroups.com
The oldest NHL list is run by Scott Pollock, and was created for all lymphoma types. This list does not have a rule against chit chat and has a very high message load. It can be of particular use to people going thru treatments or a health crisis and needing a lot of support. To subscribe, send a message to nhl-request@jubjub.wizard.com. The subject line should say SUBSCRIBE. The body of the message should read SUBSCRIBE TO NHL LIST.
Gathered by Vera Bradova © 1999-2003
Updated 9-20-2003