Subcutaneous panniculitis-like T-cell lymphoma

How is subcutaneous panniculitis-like T-cell lymphoma diagnosed?

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.

Last updated on 05-01-20

How is subcutaneous panniculitis-like T-cell lymphoma inherited?

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. 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.

Last updated on 05-01-20

What is the long-term outlook for people with subcutaneous panniculitis-like T-cell lymphoma?

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.

Last updated on 05-01-20

How many people have subcutaneous panniculitis-like T-cell lymphoma?

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.

Last updated on 05-01-20

How might subcutaneous panniculitis-like T-cell lymphoma be treated?

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.

Last updated on 05-01-20

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Freedman A, Aster JC. Clinical manifestations, pathologic features, and diagnosis of subcutaneous panniculitis-like T cell lymphoma UpToDate. Feb 2018; Reference Link Damasco F, Akilov OE. Rare Cutaneous T-cell lymphomas Hemol Oncol Clin North Am. Feb 2018; 33(1). 135-148. Reference Link 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. Reference Link

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