Researchers find strong familial tendency in rare form of lymphoma

06/07/04

NEW ORLEANS –– Scientists at Dana-Farber Cancer Institute have uncovered new evidence that Waldenstrom's macroglobulinemia – a rare form of lymphoma – can run in families. In research presented at the American Society of Clinical Oncology annual meeting, investigators documented these findings, as well as their discovery that certain markings – or antigens – on the surface of Waldenstrom's tumor cells may indicate a subtype of the disease.

Waldenstrom's is a malignancy of white blood cells called B cells, which fight disease by producing antibodies against viruses, bacteria, and other foreign substances. Usually diagnosed in people over age 65, the disease has been thought to be more prevalent in some families, but researchers don't know how frequently it occurs in such families, nor whether it takes a different form in people with a family history of the disease.

Researchers led by Dana-Farber's Steven Treon, MD, PhD, examined medical records and laboratory tests from 181 Waldenstrom's patients to look for patterns of the disease within families and to see if they could identify differences between family and non-family forms of the disease.

They found that 36 of the patients, representing 20 percent of the total, had at least one first-degree relative with either Waldenstrom's or a related B-cell disorder, such as multiple myeloma or non-Hodgkin's lymphoma. They also found that by a variety of measures – age of onset, blood counts, presence of tumor cells in bone marrow, genetic analysis, and other criteria – there were no discernible differences between the disease in patients from Waldenstrom's families and those from non-Waldenstrom's families.

In a related study slated for presentation at the ASCO conference, Treon and his colleagues examined the molecular make-up of Waldenstrom's cells to see if antigens are linked to specific varieties of the disease. They found that patients whose cells showed, or "expressed," the CD5 and CD23 antigens tended to have a greater concentration of tumor cells in their bone marrow than patients whose cancer cells lacked those antigens.

"The high incidence of familial involvement in Waldenstrom's macroglobulinemia was a surprise even to us and has given us new insights that are helping our research efforts in finding the genetic basis of this disease. It has also raised some very interesting questions from our patients who now want to know about the appropriateness of testing their family members for Waldenstrom's."

Source: Eurekalert & others

Last reviewed: By John M. Grohol, Psy.D. on 21 Feb 2009
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