A Patient's Guide to Understanding Cutaneous Lymphoma - page 18

A Patient’s Guide to Understanding
Cutaneous Lymphoma
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of CD30-positive T-cells) and may span from non-malignant, inflammatory
or infectious, all the way to full-blown lymphoma. In the family of
pcCD30+LPD, LyP is usually classified as non-malignant or as a CTCL
precursor, though some experts say it is a very low-grade form of CTCL.
This is a one-in-a-million disease. LyP’s overall prevalence is only 1.2-1.9
cases per 1 million people. That said, onset can happen at any time in
life – from early childhood to middle age, affecting both genders equally.
Black-skinned individuals seem less affected than other racial groups.
LyP is characterized by red-brown bumps and spots, which heal on their
own by turning scaly or forming a crust. Lesions can be unnoticed or itchy
and painful, taking 2-3 weeks to run their course.
This disease is not contagious, and there has been no supporting evidence
to indicate that it is genetic-based or hereditary. In fact, scientists have yet
to find a single risk factor for LyP.
The good news is that, in more than 90% of cases, LyP is a persistent but
limited disease that does not affect a patient’s overall health. There is no
known cure, but there are many treatments for living with the disease.
At the other end of the spectrum of pcCD30+LPD, anaplastic large cell
lymphoma (ALCL) is a true T-cell lymphoma, which comprises about 20%
of all CTCL, but only 3% of all non-Hodgkin’s lymphomas in adults. The
disease is characterized by the large size and misshape of the cells under
the microscope and by the uniform expression of a special marker on the
lymphoma cells called CD30.
Like MF, primary cutaneous ALCL (pcALCL) is an indolent, slow-
growing type of CTCL, with a good prognosis. Because they are part
of the same spectrum of diseases, pcALCL and LyP often are found
together in the same patient. LyP, unlike pcALCL, almost always
shows spontaneous self-healing, and is occasionally a precursor to the
development of pcALCL or other lymphomas, most commonly MF.
Characteristic features of pcALCL include single or multiple raised red
skin lesions and nodules, which are larger than those seen in LyP, do
not go away, may itch, do not typically crust and they have a tendency to
ulcerate. These lesions may appear anywhere on the body and grow very
slowly, so they may be present for a long time before being diagnosed.
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