Peripheral T-cell lymphoma, (NOS)
Peripheral T-cell lymphoma, NOS designates a heterogeneous category of nodal and extranodal mature T-cell lymphomas, which do not correspond to any of the specifically defined entities of mature T-cell lymphoma
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
. They account for approximately 30% of peripheral T-cell lymphomas (PTCL) in Western countries. Most patients are adults, with a M:F ratio of 2:1. Peripheral lymph node are typically involved, but any site may be affected and generalized disease is common.
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
International Agency for Research on Cancer: Lyon 2008
Monomorphic CD56+ intestinal T-cell lymphoma is a type of peripheral T-cell lymphomas. This is intestinal tumour of intraepithelial T-lymphocytes composed of small- to medium-sized monomorphic cytotoxic T-lymphocytes commonly expressing CD56. This lymphoma also has been referred to as Type II enteropathy-associated T-cell lymphoma (EATL). This group comprises 10–20% of gastro - intestinal T-cell lymphomas [[540, 690]]. The etiology of this disease is unknown; there is also no clear genetic link with coeliac disease. The neoplastic cells are small, round and monomorphic and immunoreactive for CD56 (NACM). The clinical course of this disease is similar to that for Type I EATL
; the prognosis for overt lymphoma is very bad owing to intestinal complications (perforation, peritonitis) or early spread with lung involvement
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
International Agency for Research on Cancer: Lyon 2010