2007.11.23 – Rare cancer in spotlight


Rare cancer in spotlight


A rare blood cancer making headlines locally is taking the national stage.

Polycythemia vera, a disease studied in Schuylkill, Luzerne and Carbon counties, will be the subject of a national meeting of blood disorder experts.

“It’s relevant because this is a group of hematologists that deal with this issue,” said Steve Dearwent, chief of investigations branch, Division of Health Studies for the Agency for Toxic Substances and Disease Registry.

The agency that near the end of October presented data showing elevated cases of the rare condition locally will be discussing the issue at the 49th annual meeting of the American Society of Hematology in Atlanta.

Federal officials are preparing a presentation for the national group at its meeting Dec. 10, confirmed Laura Stark, a communications specialist with the society.

On Oct. 24, the federal agency reported confirming 38 cases of polycythemia vera, which leads to overproduction of red blood cells, in the three-county area, which is roughly 52 percent higher than the 25 cases that might have been expected given the population.

Dearwent said he expected to be joined by Dr. Ronald Hoffman, a national expert on polycythemia vera from Mount Sinai School of Medicine, New York City, who also briefed local residents at Genetti’s Best Western Hotel and Motor Lodge, Hazleton, in October.

Local community leaders are concerned elevated levels of the disease are the result of environmental contamination, but federal officials say no cause for the disorder has yet been determined.

Dearwent said the primary purpose of the presentation planned for the national meeting in December is to encourage increased genetic testing for the blood condition to confirm diagnosis and improve data provided to disease registries.

Genetic testing leading to diagnosis was a tool in each of the 38 local cases identified, distinguishing between what experts call primary and secondary polycythemia vera, Dearwent said.

In cases of the primary condition, he said, a genetic defect is responsible for the red blood cell overproduction.

However, Dearwent said other factors like smoking, which decreases the efficiency with which the blood transports oxygen, might also lead to an overproduction of red blood cells that could be indistinguishable from the primary condition without a genetic test.

©The REPUBLICAN & Herald 2007