Subcutaneous T-cell lymphoma
Subcutaneous T-cell lymphoma (also known as a "Panniculitis-like T-cell lymphoma") is a cutaneous condition that most commonly presents in young adults, and is characterized by subcutaneous nodules.[1]:739
Classification
Subcutaneous panniculitis-like T-cell lymphoma, is a subtype of Peripheral T-cell lymphoma. Peripheral T-cell lymphoma (PTCL) is defined as a diverse group of aggressive lymphomas that develop from mature-stage white blood cells called T-cells and natural killer cells (NK cells) (see figure for an overview of PTCL subtypes). PTCL is a type of non-Hodgkin’s lymphoma (NHL).[2] NHL affects two particular types of white blood cells: B-cells and T-cells. PTCL specifically affects T-cells, and results when T-cells develop and grow abnormally.
Subcutaneous Panniculitis-like T-cell Lymphoma is a rare type of lymphoma that infiltrates the subcutaneous fat but does not involve the skin. There are two subtypes – alpha-beta and gamma-delta. Patients with the gamma-delta subtype have a more aggressive clinical course.[3]
It is described as CD3+/CD4-/CD8+, with CD30 and CD56 usually negative.[4]
Treatment
Currently Subcutaneous Panniculitis-like T-cell Lymphoma, being a PTCL subtype, is treated similarly to B-cell lymphomas. However, in recent years, scientists have developed techniques to better recognize the different types of lymphomas, such as PTCL. It is now understood that PTCL behaves differently from B-cell lymphomas and therapies are being developed that specifically target these types of lymphoma. Currently, however, there are no therapies approved by the U.S. Food and Drug Administration (FDA) specifically for PTCL. Anthracycline-containing chemotherapy regimens are commonly offered as the initial therapy. Some patients may receive a stem cell transplant.[5][6][7][8][9][10][11][12] Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation.
CHOP or CHOP-like regimens have been used.[13]
Research
Pralatrexate is one compound currently under investigations for the treatment of PTCL. For information please consult the US clinical trials database (http://www.clinicaltrials.gov).
See also
References
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- ↑ Swerdlow SH, WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 2008
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- ↑ d’Amore F, et al. Blood. 2006;108:A401
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