This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of lymphoma that mainly affects the skin. Lymphomas are cancers that result from a type of white blood cell that grows out of control. In SPTCL, people develop multiple painless nodules in the fat layer under the skin and can these cause swelling. This is known as panniculitis. The cause of this type of cancer is unknown, but genetic factors may be involved. It can be diagnosed by a skin biopsy of the affected area. Treatment for SPTCL depends on the symptoms but often involves taking a steroid medication or a medication that lessens the immune response. Some people with more aggressive SPTCL may need chemotherapy. SPTCL can occur in children or adults, but is typically diagnosed in the 30s. For unknown reasons, it occurs more often in women than men.
SPTCL is a very slow-growing cancer and does not usually spread beyond the subcutaneous fat layer. About 20% of people with SPTCL will also develop another condition called hemophagocytic lymphohistiocytosis (HLH) in which the body makes too many immune cells. These immune cells attack other cells in the body and cause an enlarged spleen and a decreased level of blood cells in the body.
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