Reviewed: November 2013 - Cutaneous Lymphoma Foundation’s Medical Advisory Committee
Conversation With A Patient: 'I Want to Help Other People'
Article previously published in the Cutaneous Lymphoma Foundation's Forum 2012 Issue 2 newsletter.
A retired dentist who is an avid cyclist discusses his leading-edge treatments and life in remission. He was diagnosed in 2005 with Stage 3 of Sezary syndrome, a form of cutaneous T-cell lymphoma (CTCL). Neil Dicker, who has spoken at two of the Foundation’s Patient Educational Forums, marked his third year of remission this past May (2012). "It can turn out this way," says Dicker, who is 64. "Never give up."
Q. What was the prognosis seven years ago?
A. Online research showed a life expectancy of about 100 months, and I accepted that. My symptoms had escalated for a year by the time I was properly diagnosed and referred to Dr. Alain Rook at the University of Pennsylvania, an outstanding CTCL specialist who's on the Medical Advisory Board of the Cutaneous Lymphoma Foundation. He described long-term success with patients receiving conventional treatment for seven to ten years.
Q. What approaches were used?
A. For three months we tried total skin electron beam radiation, a very challenging treatment. Then came a series of biological treatments that included photopheresis and later injections of Intron A and B and Leukine, along with oral Targretin. Topically, I used Carmustine ointment and all of the corticosteroid ointments.
Search for New Approach
Q. What was your life and outlook like?
A. I sold my practice and retired from dentistry in spring 2005. It was increasingly clear my condition was getting worse as the level of cancer cells in my blood increased. I was tired of seemingly endless treatment and became despondent about being chronically ill.
I began researching a stem cell transplant. In 2008, Dr. Rook referred me to Dr. Alison Loren at the Hospital of the University of Pennsylvania, who explained the process. There were no family matches, although I have four sisters, but the hospital's transplant coordinator found several potential donors.
Q. What was the turning point that followed?
A. I agreed to the transplant process after a year of soul-searching and discussions with my wife Linda and our four children. In August 2009, stem cells were infused into my body – a 20-minute procedure on the sixth day of a 22-day hospitalization. Linda and I then lived part-time in a Philadelphia apartment for several months. There were tense situations, but I never had to be hospitalized again. Over time, I felt stronger. And I was in remission!
Q. How long did that status continue?
A. Just over half a year. By spring 2010, tests showed the transplanted cells weren't engrafting properly. It was clear things were not 100 percent, though I resumed biking and other activities.
That fall, tests showed the transplant might be failing, although I was still in remission.
'The Ultimate Gift'
Q. You and the doctors weren't out of options, right?
A. Definitely not. That November I received a DLI [donor lymphocyte infusion] from the same donor, whose identity I still didn't know. An anonymous stranger had given me the ultimate gift of time and life.
I was excited, but also very afraid of the DLI. It could fully integrate the transplant, but carried the risk of life-threatening GVHD – graft-versus-host disease. Both occurred.
Within six weeks, stem cell integration went from 48 percent to 100 percent. I became very ill from GVHD, but wasn't hospitalized. Strong corticosteroids and immunosuppressant drugs, good physicians, family support and good luck all brought recovery. It was heads or tails for a while, and the coin landed up for me.
I also hit the lucky odds another way. The Gift of Life Bone Marrow Foundation picked me to meet my donor at a fund-raising dinner in New York. Last May on center stage at the Grand Hyatt, Linda and I met Arielle, the 26-year-old woman who volunteered for an international genetic registry and whose stem cells saved my life. Without her, we wouldn't be talking.
Active 2012 Travel Pace
Q. How are you doing now?
A. The transplant is 100-percent integrated and there is no sign of cancer. My skin is completely clear and I have no CTCL symptoms. I'm off all medication for this disease and transplant-related issues.
I enjoy every day. This summer I'll ride for five days in an event called Bike Virginia and will travel to Lake Placid, N.Y. In October my wife and I will go to Paris to celebrate our 25th anniversary and my 65th birthday.
Q. How have you changed?
A. I've traveled from being a cancer patient with a very questionable prognosis to a person who is cancer-free. That's why I want to help other people through the Cutaneous Lymphoma Foundation by talking about the stem cell option, which isn’t well-known. I encourage anyone who is a candidate to consider it.
In the Tour de France, a polka dot jersey is awarded to the best hill climber. His qualities include perseverance, persistence and courage. I have my own polka dot jersey, and although I didn’t win it in a race I'm still proud of it. I fought my cancer with those same qualities and I encourage all those with CTCL or any cancer to do the same.
Stay positive, stay strong and seek out the best medical advice you can find.
Dr. Neil Dicker welcomes inquiries from anyone seeking a patient's perspective. He can be contacted at firstname.lastname@example.org
The two most common types of cutaneous T-cell lymphomas (CTCL) are mycosis fungoides, which is often indolent, appears as reddish skin patches and can progress over many years, and an advanced and leukemic form of mycosis fungoides called Sézary syndrome. Sézary syndrome is distinguished from mycosis fungoides by the presence of malignant lymphocytes in the blood and is characterized by extensive thin red, itchy rashes covering over 80 percent of the body. In some cases, thicker, red patches (or plaques) and tumors may also appear. In addition, these symptoms may be accompanied by changes in the nails, hair or eyelids or the presence of enlarged lymph nodes.
There are about 3,000 new cases of mycosis fungoides each year in the U.S. and approximately 15 percent of those are diagnosed as Sézary syndrome. Although this type of NHL can affect people of any age, Sézary syndrome usually occurs in adults ages 50 and over and is slightly more common in men than women. There are no known risk factors for this type of cutaneous T-cell lymphoma.
Many of the same procedures used to diagnose and stage other types of cutaneous T-cell lymphomas are used in Sézary syndrome, including a physical exam and history; blood tests to identify antigens, or markers, on the surface of the cells in the blood; a skin and/or lymph node biopsy (removal of a small piece of tissue) for examination under the microscope by a pathologist (a doctor who studies tissues and cells to identify diseases); and a series of imaging tests such as CT (computerized axial tomography), MRI (magnetic resonance imaging) and/or PET (positron emission tomography) scans to determine if the cancer has spread to lymph nodes or other organs. In addition to these diagnostic tests, occasionally a bone marrow biopsy may be necessary to verify complete staging.
Because mycosis fungoides and Sézary syndrome are such rare cancers, it is important to confirm a diagnosis by a dermatopathologist or a hematopathologist, a pathologist who is an expert in diagnosing lymphomas.
The following staging system is used to determine the extent of mycosis fungoides:
Less than 10 percent of the skin is covered in red patches and/or plaques.
Ten percent or more of the skin surface is covered in patches and/or plaques.
Any amount of the skin surface is covered with patches and/or plaques; lymph nodes are enlarged, but the cancer has not spread to them.
One or more tumors are found on the skin; lymph nodes may be enlarged, but the cancer has not spread to them.
Nearly all of the skin is reddened and may have patches, plaques or tumors; lymph nodes may be enlarged, but the cancer has not spread to them.
Most of the skin area is reddened and there is involvement of the blood with malignant cells or any amount of the skin surface is covered with patches, plagues or tumors; cancer has spread to the lymph nodes and the lymph nodes may be enlarged.
Most of the skin is reddened or any amount of the skin surface is covered with patches, plaques or tumors; cancer has spread to other organs; and lymph nodes may be enlarged whether cancer has spread to them or not.
Common medications include:
- Alemtuzumab (Campath), a monoclonal antibody
- Bexarotene (Targretin), a retinoid
- Gemcitabine (Gemzar), an antimetabolite chemotherapy
- Interferon alfa or interleukin-2, immune stimulants that bind to specific cell-surface receptors
- Liposomal doxorubicin (Doxil), a chemo therapy that binds to DNA
- Methotrexate (Trexall), an antimetabolite chemotherapy, which blocks the metabolism of cells
- Vorinostat (Zolinza), a histone deacetylase inhibitor
Common combination therapies include:
- Bexarotene (Targretin) and interferon alpha
- Bexarotene and phototherapy
- ECP (extracorporeal photopheresis) and bexarotene
- ECP and interferon alpha ECP, interferon alpha and bexarotene
- Phototherapy and interferon alpha
Some second-line chemotherapies for relapsed (the recurrence of disease) or refractory (disease that is resistant to treatment) patients include:
- Bexarotene (Targretin) and denileukin difitox (Ontak)
- Chlorambucil (Leukeran)
- Cyclophosphamide (Cytoxan)
- Pentostatin (Nipent)
Clinical trials are crucial in identifying effective drugs, prognostic strategies and determining optimal doses for lymphoma patients. Because mycosis fungoides and Sézary syndrome are such rare diseases, finding enough patients to enroll in clinical trials is often difficult. If you are interested in participating in a clinical trial, talk to your doctor about an appropriate trial for you.
Complementary and alternative medicines are nonstandard therapies that may help patients cope with their cancer and its treatment, but that should not be used in place of standard treatment. No alternative therapy has ever been proven effective against lymphoma. However, complementary therapies such as meditation, yoga, acupuncture, exercise, diet and relaxation techniques have been shown to be effective in combating some treatment side effects. Before embarking on any complementary therapies, patients should discuss the matter with their healthcare team. Certain unproven treatments, including some herbal supplements, can interfere with standard lymphoma treatments or may cause serious side effects.
It is important for patients both during and after treatment to be proactive in their healthcare, including keeping a master file of medical records, asking questions, reporting new symptoms, exercising and eating a balanced diet. In addition, patients who smoke should strongly consider stopping. Follow-up visits for people with Sézary syndrome often depends on the stage of the disease and treatment and can range from as frequently as every few weeks when starting new therapies that require monitoring to as little as every six months.
Typically, follow-up visits include physical examinations, blood tests and occasionally imaging tests such as CT or PET scans. Besides determining disease recurrence, follow-up care can help identify and resolve unusual side effects of treatment.