National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Subcutaneous panniculitis-like T-cell lymphoma


Other Names:
SPTCL
Categories:
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of lymphoma that mainly affects the skin.[1][2] 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.[3] 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.[1] 

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)[3] 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. 
Last updated: 1/30/2019
It is not clear if subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is inherited in families.

One research study has suggested the SPTCL is associated with gene mutations in the HAVCR2 gene. In families with mutations in this gene, SPTCL was inherited in an autosomal recessive pattern.[3] In autosomal recessive inheritance, both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition. The parents are known as "carriers". Autosomal recessive disorders are typically not seen in every generation of an affected family.
 
In these families, SPTCL associated with HAVCR2 gene mutations was more likely to include hemophagocytic lymphohistiocytosis.[3] 

Last updated: 1/30/2019
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is diagnosed based on the symptoms and patient’s history. In addition, a skin biopsy that includes the underlying tissue is examined for features in the cells typical for SPTCL. Sometimes, more than one skin biopsy is necessary to make the diagnosis.  Additional blood work may be done to check for conditions that can look like SPTCL.[1] 
Last updated: 1/30/2019
Many people with subcutaneous panniculitis-like T-cell lymphoma (SPTCL) can be treated with oral corticosteroids drugs and/or other drugs that suppress the immune system, such as methotrexate, cyclosporine or bexarotene. Some people may need chemotherapy and may be treated with a combination of cyclophosphamide, doxorubicin, vincristine and prednisone, known as CHOP chemotherapy.[1][2]
Last updated: 1/30/2019
Most people who develop subcutaneous panniculitis-like T-cell lymphoma (SPTCL) have a good prognosis. About 80% of people with this cancer are still alive 5 years after they are first diagnosed. Some cases of SPTCL go away on their own. The diagnosis of hemophagocytic lymphohistiocytosis (HLH) along with SPTCL is an indication of a more aggressive cancer and may influence long-term outcome.[1]
Last updated: 1/30/2019
The exact prevalence of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is unknown. It is a rare cancer and only about 1% of all types of cutaneous lymphomas are found to be SPTCL. Women are more likely to be diagnosed than men, and the average age of diagnosis is in the mid-30s. Both children and adults can develop SPTCL.[1]
Last updated: 1/30/2019

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Subcutaneous panniculitis-like T-cell lymphoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Subcutaneous panniculitis-like T-cell lymphoma. Click on the link to view a sample search on this topic.

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  1. Freedman A, Aster JC. Clinical manifestations, pathologic features, and diagnosis of subcutaneous panniculitis-like T cell lymphoma. UpToDate. Feb 2018; www.uptodate.com/contents/clinical-manifestations-pathologic-features-and-diagnosis-of-subcutaneous-panniculitis-like-t-cell-lymphoma.
  2. Damasco F, Akilov OE. Rare Cutaneous T-cell lymphomas. Hemol Oncol Clin North Am. Feb 2018; 33(1):135-148. https://www.ncbi.nlm.nih.gov/pubmed/30497671.
  3. Gayden T, Sepulveda FE, Khuong-Quang DA, Pratt J, et al. Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome. Nat Genet. Dec 2018; 50(12):1650-1657. https://www.ncbi.nlm.nih.gov/pubmed/30374066.