Mayo Clinic treats rare cancer with high-dose chemotherapy and stem-cell transplant

Wednesday, March 29, 2006

JACKSONVILLE, Fla. — When Cynthia Farmer, a 53-year-old single mother of three, talks about POEMS, she's not referring to her favorite verses from Robert Frost. She's identifying her rare blood disorder, which according to Mayo Clinic physicians, may be treated with high-dose chemotherapy and an autologous hematopoietic stem cell transplant. Having seen at least 100 cases, Mayo Clinic physicians have significant experience treating patients with POEMS syndrome.

While this form of cancer is rare, that Farmer contracted it is even rarer. The first signs of her illness came in 1986, when she was 33 years old. Median age of POEMS patients is 51, and about 60 percent are male. It is neither inherited nor contagious. Many of the symptoms, such as fatigue, numbness, aches, fever and diarrhea, mimic other more common diseases, and patients don't necessarily seek treatment early on.

Farmer's principal physician, Mayo Clinic oncologist Dr. Gerardo Colon-Otero, says because POEMS is so rare many physicians are not aware of it. "They've never seen someone with it," he says. "There aren't too many papers written about it either. So many patients we see have gone undiagnosed for many years."

Farmer first sought help in the late 1980s in Virginia, but her disease was not diagnosed. Her health deteriorated to the point that she was legally blind. At the advice of her physician, she came to Mayo Clinic in Jacksonville, Fla., for a second opinion. In 1990, she met with Colon-Otero, who immediately recognized her symptoms as POEMS. It was the first time Farmer had heard of the disease. POEMS is an acronym for the disease's five most common symptoms. The "M" or monoclonal gammopathy refers to a certain abnormal protein that is present in the blood.

Although several experimental treatments followed Farmer's diagnosis, her quality of life continued to decline. "I couldn't hold a pen to write my name, I couldn't walk, I couldn't feed myself," she says. Then Colon-Otero suggested another experiment — high-dose chemotherapy followed by a stem cell transplant. Cells used would be harvested from Farmer's own body, known scientifically as an autologous stem cell transplant.

Though intended to destroy cancerous cells, high doses of chemotherapy also will damage normal bone marrow cells and bone marrow 'seed' cells, which are also known as hematopoietic stem cells. These 'seed' or hematopoietic stem cells are vital since they are the source of blood cells that carry oxygen, fight infection and prevent bleeding. Before receiving high-dose chemotherapy to destroy the cells that make the abnormal proteins, doctors harvested Farmer's own blood-forming stem cells. After the therapy, these seed cells were returned to Farmer via a transplant so that her bone marrow could grow back and produce blood cells.

"Whether any of the POEMS patients we've treated with a stem cell transplant will be cured or not is still unknown," says Colon-Otero, "but most of the patients who have received the transplant have improved dramatically." The Mayo Clinic POEMS transplant experience — the largest published series in the world — was led by Mayo physicians Angela Dispenzieri and Alvaro Moreno-Aspitia. Though treatment for POEMS is not standardized, Mayo Clinic physicians advise that high-dose chemotherapy and stem cell transplantation should be considered a therapeutic option for patients with POEMS. This therapy is not for all patients as it is dependent on their symptoms.

Farmer improvement has been dramatic. Since the transplant, she's earned a second master's degree, is looking for a job as a rehabilitation counselor, has regained limited mobility through a walker and is enjoying her new role as a grandmother.

Mayo Clinic is a leader in POEMS research and diagnosis. Patients are treated by a multidisciplinary team of physicians, including neurologists, endocrinologists, radiation oncologists, physical therapists and others.

POEMS is an acronym for the disease's five most common symptoms:

• Polyneuropathy: affecting the nerves mostly through numbness of the extremities
• Organomegaly: enlarging the organs, usually the liver, spleen or lymph nodes
• Endocrinopathy: attacking certain glands that
• Monoclonal gammopathy: referring to a certain abnormal protein that is often present with this disease and
• Skin defects: including excessive darkening/reddening of the skin.

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