Abstract
The extranodal T-cell lymphomas comprise approximately 10 % of aggressive T-cell lymphomas. Defined largely by their tissue trophism, these disorders have generally been associated with a poor outcome after conventional therapeutic approaches. The panniculitis-like T-cell lymphomas may be associated with inflammatory panniculitis or autoimmune disorders and often have an excellent prognosis, while the gamma delta cytotoxic T-cell lymphomas of the skin have a poor outcome. Hepatosplenic T-cell lymphomas are often associated with isochromosome 7q and have been associated with immunosuppressive therapy, while the enteropathy-associated T-cell lymphomas are comprised of two morphologic types, one of which arises in the setting of celiac sprue. Treatments for these rare T-cell lymphomas are not well defined, but newer agents have shown promise.
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Lansigan, F., Hudnall, S.D., Foss, F. (2013). Rare Subtypes of Extranodal T-Cell Lymphoma. In: Foss, F. (eds) T-Cell Lymphomas. Contemporary Hematology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-170-7_7
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DOI: https://doi.org/10.1007/978-1-62703-170-7_7
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