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Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell non-Hodgkin lymphoma (NHL). It is often mistaken for panniculitis, an inflammation of the fatty (subcutaneous) tissue in the body. This is because SPTCL causes nodules to form in the fatty tissue.
SPTCL occurs in all age groups.
With SPTCL, nodules form under the skin in the subcutaneous tissue (subcutaneous nodules). Usually, the nodules occur in the legs or trunk of the body. These can become open and inflamed.
People with this type of lymphoma can also develop a very serious condition where certain parts of the immune system are activated (hemophagocytic syndrome). Hemophagocytic syndrome causes:
It was thought that there were 2 subtypes of SPTCL:
The alpha/beta subtype (SPTCL-AB) is now considered the only type of subcutaneous panniculitis-like T cell lymphoma. It is a slow-growing (indolent) disease with a favourable prognosis.
The gamma/delta subtype (SPTCL-GD) is included in the cutaneous T-cell lymphoma group and is a more aggressive type. Hemophagocytic syndrome is most often associated with SPTCL-GD and has a less favourable prognosis.
In most cases, SPTCL is naturally aggressive, but is responsive to chemotherapy. A few cases are indolent.
Treatment of SPTCL varies widely because it is not yet certain how to best treat this uncommon type of lymphoma. Chemotherapy, corticosteroids or radiation therapy may be used.
Combinations of chemotherapy drugs, mainly CHOP or CHOP-like therapies, are usually given to treat SPTCL.
Corticosteroids, such as prednisone, or drugs that suppress the immune system, like methotrexate or cyclosporine (Neoral), have also been used in people with less aggressive SPTCL. They have proven to control SPTCL for a long period of time.
External beam radiation therapy has also been used to treat SPTCL.
A stem cell transplant (SCT) may be offered to some people with SPTCL whose lymphoma no longer seems to be responding to treatment or whose lymphoma recurs.
For more detailed information on specific drugs, go to sources of drug information.
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