Mycosis Fungoides (MF) is the most common variant of a rare form of lymphoma known as
Cutaneous T-Cell Lymphoma, often referred to as CTCL for short. Its initial
primary symptoms include red, itchy patches of skin that appear similar to
psoriasis or eczema. Consequently, MF is often ignored by those with
symptoms and then frequently misdiagnosed by physicians. The cause of CTCL
is unknown. There is no known cure.
In technical terms, mycosis fungoides is a non-Hodgkins lymphoma composed of malignant T
lymphocytes of the CD4 phenotype (1). It is a rare condition with an incidence
rate reportedly ranging from 2.9 per million people (2) to 4.2 cases per
million. (1)
Most people with CTCL are between 40 and 60 years old at the time of diagnosis (1).
However, most people have symptoms that have persisted for many years before
being properly diagnosed. The median duration of skin symptoms before
diagnosis is 6 years (3). CTCL is twice as common in men as it is in women (3)
and
almost twice as common in black people than white (1) (incidence ratio 1.6:1.0)(3).
The annual incidence rate in the US is about twice that in Eastern Europe,
although this might be a consequence of awareness levels (2).
The median survival time for patients with CTCL, regardless of stage of
disease, is 8 to 10 years (4). Over 50% of patients die within 10 years of
diagnosis (5). Over 50% of deaths from CTCL are caused by Staphylococcus aureus
or Pseudomonas aeruginosa sepsis (1), a consequence of the impaired immune system
resulting from CTCL. About 10% of people diagnosed with MF will experience
a progression to lymph node or internal involvement (6). Patients with visceral
or lymph node involvement have a median
survival of less than 3 years (4) with some reports indicating a survival of one
year (5). CTCL tumors can undergo a transformation into a large-cell variant of
CTCL, which results in an extremely poor prognosis. (1)
A variant of mycosis fungoides is follicular mycosis fungoides. This condition
is increasingly being considered a separate disease entity due to some distinctive
features as well as its resistance to treatment and lower survival rates.(7,8).
As you can see, most of the references cited on this page are web-based. There
is extensive information on this disease, its treatment, and ongoing research
available on the web. Simply go to Google (9) or Yahoo (10) and type CTCL (or
the full name in words) and you will find a wealth of information. You might
also do your search specifically on Mycosis fungoides (MF) - another term often used when discussing
CTCL. There is also a strong association between follicular mucinosis and CTCL/MF,
so interested persons might search on this disorder as well. The term follicular
mycosis fungoides is often used to distinguish this variant from mycosis
fungoides.
I will add an
editorial note here. There are two general categories of information
available on the web concerning CTCL. The first is the type for popular
consumption and meant to prevent alarm. These sites take the non-threatening
tone - don't worry, be
happy, you won't die from this disease. The second gets down to the technical
details and facts, which are not nearly so rosy. If you have this disease, I
hope the first of these categories will be most accurate for you.
Lastly, I should add that I am not a doctor and the information I have presented
here has been filtered through the eyes of a non-medical layman.
1.
http://www.moffitt.usf.edu/pubs/ccj/v5n1/article1.html
2.
http://www.healthatoz.com/healthatoz/Atoz/ency/cutaneous_t-cell_lymphoma.html
3.
http://www.emedicine.com/MED/topic1541.htm
4.
http://www.ctclconsult.com/module_2.htm
5.
http://www.mcevoy.demon.co.uk/Medicine/Pathology/Haematology/WBC_Diseases/SALT.html
6.
http://www.mffoundation.org/fastfacts/MF_fastfacts.html
7.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11004619&dopt=Abstract
8. Arch Dermatol/Vol 138, Feb. 2002.
9.http://www.google.com/
10.http://www.yahoo.com/
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