Rare illness is a complex enigma
Cause and cure of disease that struck lawmaker are elusive.
By Dorsey Griffith -- Bee Medical Writer
Published January 4, 2005
Robert T. Matsui
The disease that led to the death Saturday of Rep. Robert
Matsui is a rare and complex illness, which in many cases has no
known cause and no sure cure.
Matsui had been diagnosed a few months ago with a form of a
blood disorder called myelodysplastic syndrome, or MDS.
He was admitted to a Washington, D.C.-area hospital on
Christmas Eve with pneumonia and died eight days later from the
infection.
Matsui's office has not released additional details about his
illness or any treatment he had received, so experts are
reluctant to speculate about the lawmaker's particular struggle
with the disease.
"Anybody who comes into the hospital with pneumonia can
die of pneumonia, especially when they are over age 60,"
said Dr. Joseph Tuscano, an oncologist and associate professor
of medicine at UC Davis.
"The fact that he already had a suppressed
immune system really puts him at elevated risk of
dying."
What is known is that MDS is really a constellation of
disorders, some of which can progress to acute myeloid leukemia,
a blood disease in the cancer family.
In MDS, a person's bone marrow does not produce enough
blood cells.
Working normally, the bone marrow makes three major types of
blood cells: red blood cells transport oxygen from the lungs to
cells throughout the body; white blood cells help the body fight
infections; and platelets help clot the blood and prevent
bleeding.
Symptoms of the disease include a change in the blood count,
anemia, weakness, fatigue, frequent infections, easy bruising,
bleeding, fever and weight loss.
An estimated 7,000 to 12,000 new cases of the disease are
diagnosed every year in the United States, typically in people
over age 60, according to the Myelodysplastic Syndromes
Foundation.
In rare cases, MDS can be caused by exposure to certain
chemicals, including chemotherapy drugs. Patients being treated
for Hodgkin's lymphoma, for example, are at risk for MDS.
Exposure to radiation, either for treatment of a disease or
from radioactive weapons, also has been associated with MDS, as
has exposure to high concentrations of benzene from tobacco
smoke and gasoline.
Most MDS patients, however, have no known risk factors, said
Dr. John Bennett, a leading authority on MDS at the University
of Rochester in New York.
"Ninety-plus percent of patients have no history
whatsoever that would suggest a risk factor, no significant
smoking or occupational exposure or a history of another
malignancy such as Hodgkin's lymphoma," he said.
Tuscano of UC Davis said early-stage MDS patients can live
with their disease for many years, treated with blood
transfusions, blood products and chemotherapy.
"With relatively close monitoring, the majority of
patients really do well," Tuscano said.
The higher risk patients, however, are those close to
developing leukemia and typically survive no more than two years
after being diagnosed, he said.
Only one drug has been approved specifically to treat
patients with advanced MDS. Vidaza, approved by the Food and
Drug Administration in May 2004, is considered a novel approach
because it activates genes that allow normal cell growth.
One risk of Vidaza, said Tuscano, is that it causes blood
counts to drop even lower initially, further increasing the risk
of infection or bleeding.
The only possible cure for MDS is a bone marrow transplant,
but the procedure is usually reserved for patients under age 60,
experts said. Matsui was 63.
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