2008.11.21 – Area victims of rare cancer can join study

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Area victims of rare cancer can join study

People in Schuylkill, Carbon and Luzerne counties diagnosed with the blood cancer polycythemia vera soon will be able to participate in an international research program.

BY KENT JACKSON
STAFF WRITER
kjackson@standardspeaker.com
Published: Friday, November 21, 2008 4:14 AM EST

The Geisinger Hazleton Cancer Center is completing an application so the patients will be included in the work of the Myeloproliferative Disorder Research Consortium, said Dr. Paul Roda, a Hazleton cancer specialist.

Roda helped focus attention on the incidence of polycythemia vera among local residents and said he joined Geisinger partly to obtain the resources needed to link with the consortium.

While polycythemia vera is rare — striking two of 100,000 people, according to the Merck Manual — a federal study found 33 people currently have the disease between McAdoo and Tamaqua. Four of those cases are along Ben Titus Road, which is near the McAdoo Associates site that underwent a cleanup through the federal Superfund program in the 1980s because of environmental contamination.

The incidences were statistically significant, according to a two-year study that the U.S. Agency for Toxic Substances and Disease Registry completed in August. The study also found smaller clusters of polycythemia vera near Jim Thorpe and south of Frackville.

Through the consortium, patients can contribute tissue samples that will help researchers study polycythemia vera, for which the cause is unknown.

Patients produce too many red blood cells, can show symptoms such as headache, fatigue and dizziness, and become susceptible to blood clots, strokes and heart attacks. They have blood drained regularly as a treatment.

Patients who don’t respond to that treatment will be eligible to participate in clinical trials of other treatments, Roda said.

“This is not going to happen overnight,” said Roda, adding that paperwork still has to be completed for the application to the consortium.

For the consortium’s tissue study, patients will be asked to contribute samples of blood, toenail clippings, spleen cells and bone marrow.

Their samples will be compared to polycythemia vera patients who live elsewhere.

“If we can acquire sufficient number of tissues, I think we’d want to look at the genetic differences between people who have the … disorders in the Tamaqua area and outside the Tamaqua area to see if there are any genetic differences,” said the consortium’s principal investigator, Dr. Ronald Hoffman of Mount Sinai Medical Center in New York.

Hoffman said the consortium previously obtained a grant from the National Cancer Institute, but seeks additional federal funding to further research.

A panel of polycythemia vera experts met Aug. 25 and proposed 11 research projects, some of which pertained specifically to northeastern Pennsylvania.

One proposal for a toxicological study recommended looking for signs of polycyclic aromatic hydrocarbons — by-products of coal plants and the Superfund site — in patients and healthy people to evaluate if those chemicals play a role in the Tamaqua area cluster.

A mutation in the JAK 2 gene occurs in most patients, so one proposal was to test samples from local blood banks for the mutation. Through the blood tests, researchers might spot cases of the disease before people show symptoms. The results also could be compared to other regions to determine whether the mutation occurs more often in the Tamaqua cluster than in other regions.

Studies, too, were proposed to find risk factors for polycythemia vera and determine whether the disease progresses differently in northeastern Pennsylvania than in other regions.

In June, U.S. Sen. Arlen Specter, a Republican, said the Appropriations Committee to which he belongs approved $262,000 for Drexel University to study the cluster of polycythemia vera cases in northeastern Pennsylvania.

The money will be available next year, but Specter told patients and doctors during a meeting Oct. 6 in Hazleton that he wants the National Cancer Institute to make money available sooner.

“The McAdoo, Pennsylvania, cluster may provide a unique opportunity to delve into the pathogenesis of this disease,” he wrote on Oct. 21 in a letter to the director of the institute.

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