I am certain that your friend's condition is Sézary (or Sezary)
Syndrome, a rare blood cancer. I've gathered some material from the
Web to help give you some insight into this disease and its treatment.
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Here are several excerpts from an excellent article about this
condition. For copyright reasons, I cannot post all the text; for
in-depth information, I suggest that you read the entire article.
"Mycosis fungoides and the Sezary syndrome usually develops slowly
over many years. In the early stages, the skin may itch, and dry, dark
patches may develop on the skin. As the disease gets worse, tumors may
form on the skin, a condition called mycosis fungoides. As more and
more of the skin becomes involved, the skin may become infected. The
disease can spread to lymph nodes or to other organs in the body, such
as the spleen, lungs, or liver. When large numbers of the tumor cells
are found in the blood, the condition is called the Sezary syndrome...
Once mycosis fungoides and the Sezary syndrome is found, more tests
will be done to find out if cancer cells have spread to other parts of
the body. This is called staging. A doctor needs to know the stage of
the disease to plan treatment...
There are treatments for all patients with mycosis fungoides and the
Sezary syndrome. Three kinds of treatment are commonly used:
radiation therapy (using high-energy rays to kill cancer cells)
chemotherapy (using drugs to kill cancer cells)
phototherapy (using light plus special drugs to make the cancer cells
more sensitive to the light)
Biological therapy (using the body's immune system to fight cancer) is
being tested in clinical trials.
Radiation therapy uses high-energy rays to kill cancer cells and
shrink tumors. In cutaneous T-cell lymphoma, special rays of tiny
particles called electrons are commonly used to treat all of the skin.
This is called total skin electron beam radiation therapy, or TSEB
radiation therapy. Electron beam radiation may also be given to
smaller areas of the skin. This kind of radiation only goes into the
outer layers of the skin. Another type of radiation uses x-rays to
kill cancer cells. The x-rays are usually directed to only certain
areas of the body, but there are studies using x-rays directed at the
whole body (total body irradiation).
Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be
taken by pill, or it may be put into the body by a needle in a vein or
muscle. Chemotherapy given in this way is called a systemic treatment
because the drug enters the bloodstream, travels through the body, and
can kill cancer cells throughout the body. In cutaneous T-cell
lymphoma, chemotherapy drugs may be given in a cream or lotion put on
the skin. This is called topical chemotherapy.
Phototherapy uses light to kill cancer cells. A drug that makes cancer
cells sensitive to light is given to the patient and then a special
light is used to shine on the cancer cells to kill them. In one type
of phototherapy, called PUVA therapy, a patient will receive a drug
called psoralen, and then ultraviolet A light will be shone on the
skin. In another type of phototherapy, called extracorporeal
photochemotherapy, the patient will be given drugs, and then some of
the blood cells will be taken from the body, put under a special
light, and put back into the body. If phototherapy is given,
directions from the doctor should be followed as to the amount of
sunlight the patient should receive.
Biological therapy tries to get the body to fight cancer. It uses
materials made by the body or made in a laboratory to boost, direct,
or restore the body's natural defenses against disease. Biological
therapy is sometimes called biological response modifier (BRM) therapy
or immunotherapy. Bone marrow transplantation is used to replace the
bone marrow with healthy bone marrow."
CancerWeb: Mycosis fungoides/Sezary syndrome
http://cancerweb.ncl.ac.uk/cancernet/210830.html
If you'd like more information about phototherapy, mentioned in the
article above, you may be interested in this answer I gave to another
question recently:
http://answers.google.com/answers/main?cmd=threadview&id=198377
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"Sézary syndrome is the name given when T-cell lymphoma affects the
skin of the entire body. It is also known as the red man syndrome
because the skin is bright red. The skin is also thickened, dry or
scaly and usually very itchy. Examination of the blood usually reveals
the presence of Sézary cells... The diagnosis of cutaneous T-cell
lymphoma is made by a dermatopathologist as there are characteristic
microscopic changes seen on skin biopsy. The diagnosis is often
delayed for months or years and may require several biopsies, as early
cutaneous T-cell lymphoma can be difficult to tell apart from other
skin conditions, particularly eczema. Enlarged lymph nodes may also be
biopsied. Cutaneous T-cell lymphoma can cause harmless swelling, known
as 'reactive' or 'dermopathic' lymphadenopathy, or result in malignant
growth in the lymph nodes. The blood count is normal in most patients
with cutaneous T-cell lymphoma, but an elevated white cell count is
characteristic of Sézary syndrome. Patients with advanced cutaneous
T-cell lymphoma may have CT or MRI scans to determine whether the
disease affects internal organs... Cutaneous T-cell lymphoma may
remain confined to the skin for many years, but the abnormal cells may
eventually infiltrate other tissues including blood, lymph nodes,
lungs, heart, liver and spleen. Unlike some other lymphomas, the
outlook is generally good. Symptoms can usually be controlled with
treatment. However treatment is not curative."
Dermnet, New Zealand Dermatological Society: Cutaneous T-cell lymphoma
http://www.dermnetnz.org/index.html
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"Cutaneous T-cell lymphoma (mycosis fungoides, Sezary syndrome): This
T-cell lymphoma starts in the skin. It is rare, making up less than 1%
of all lymphomas. Most patients are in their 50s or 60s. This lymphoma
usually begins as patchy, scaly, red lesions on the skin. They then
progress to more solid, raised tumors that can become bigger and
mushroom-like (so first named mycosis fungoides). In time, the
lymphoma can invade lymph nodes and then organs like the liver and
spleen. The growth rate varies among patients.
In the Sezary syndrome, the lymphoma cells are found in the blood.
Here the skin is involved all over instead of in patches. It usually
appears thickened and very red, and it is often itchy. Survival
depends on whether the lymphoma spreads. In Sezary syndrome the
lymphoma has spread all over the body through the blood. Patient
survival at 5 years ranges from 58% for slow growing lymphomas to 5%
for faster growing ones."
American Cancer Society: What Is Non-Hodgkin's Lymphoma?
http://www.cancer.org/docroot/CRI/content/CRI_2_4_1X_What_Is_Non_Hodgkins_Lymphoma_32.asp
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"Sezary Syndrome: This is a variant of a cutaneous T-cell lymphoma. It
likely represents the leukemic phase of mycosis fungoides. The typical
patient is an older adult with generalized erythroderma [skin
redness], pruritis (itching), and Sezary cells circulating in the
peripheral blood. This Sezary cell is the malignant pleomorphic T cell
seen in mycosis fungoides and has a convoluted nucleus... The Sezary
syndrome is a late and ominous development in mycosis fungoides and
may progress to extensive organ involvement. In general, the higher
the Sezary count, the poorer the prognosis."
The Doctor's Doctor: Sezary Syndrome
http://www.thedoctorsdoctor.com/diseases/sezary_syndrome.htm
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Here is an article that discusses several treatment options:
Dermatology Online Journal: Current treatment of Cutaneous T-Cell
Lymphoma
http://dermatology.cdlib.org/DOJvol7num1/transactions/mycosis_fungoides/duvic.html
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You may be interested in a recently-published book, "The Official
Patient's Sourcebook on Mycosis Fungoides and the Sezary Syndrome: A
Revised and Updated Directory for the Internet Age":
Amazon.com
http://www.amazon.com/exec/obidos/ASIN/0597834644/
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I hope I've been able to provide some useful info here. As a person
who has struggled for many years with a chronic illness which is
treatable but not curable, I can empathize with your friend. Best
wishes to both of you.
If you are interested in learning more on the Web, these are the
search terms that gave me the best results:
"sezary syndrome"
"sézary syndrome"
"mycosis fungoides"
"cutaneous t-cell lymphoma"
Do keep in mind that Google Answers is not an authoritative source of
medical advice, and the material above is for informational purposes
only. If I have misidentified the medical condition, if you have a
question, or if anything I've said is unclear, please request
clarification; I'll gladly offer further assistance before you rate my
answer.
Best regards,
pinkfreud |
