|
Cutaneous T-cell lymphomaCutaneous T-Cell Lymphoma (CTCL) as the name suggests is a type of lymphoma in which T-cells accumulate in the skin. The reason for this is because the T-cells involved are the ones which normally patrol the skin.
The skin is the second most common extranodal site for lymphoma; gastrointestinal sites are first. Of all primary cutaneous lymphomas, 65% are of the T-cell type. The most common immunophenotype is CD4 positive.
A diagnosis of CTCL is not in itself a final diagnosis. It comes in a variety of forms which have a different prognosis, and different behaviour and response to treatment. Here is a quick summary of the major types:
In the Indolent varieties we have:
Mycosis Fungoides / Sézary Syndrome or (Patient
version)
Long term survival prognosis for MF/SS depends largely on the stage at diagnosis. Early stage patients have good prospects for survival of 20 years or more. Those who present at late stage disease have poor prognosis for 5 year survival.
MF/SS is a very difficult disease to diagnose. In the early stages it usually presents as a skin rashes consisting of patches, plaques or tumours. Even when a biopsy is done at this stage it often will not show MF/SS and is often identified as a benign skin condition. On average, when discovered in the early stages it will take up to six years to obtain a final diagnosis. Repeated biopsies will frequently yield no diagnosis. This is why it is often not diagnosed until the later stages. (not unlike many other indolent varieties of lymphoma)
Other Mycosis Fungoides / Sézary Syndrome resources
|