T-cell lymphomas


mature T-cell neoplasms

A group of neoplasms defined by the WHO classification (first published in 2001 and updated in 2008, which draws their parentage from the REAL (Revised European American Lymphoma) classification), which groups lymphomas by cell type (i.e., the normal cell type that most resembles the tumour) and defining phenotypic, molecular or cytogenetic characteristics.
Mature T cell neoplasms
▪ T-cell prolymphocytic leukaemia.
▪ T-cell large granular lymphocytic leukaemia.
▪ Adult T-cell leukaemia/lymphoma.
▪ Extranodal NK/T cell lymphoma, nasal type.
▪ Enteropathy-type T-cell lymphoma.
▪ Hepatosplenic T-cell lymphoma.
▪ Mycosis fungoides (Sezary syndrome).
▪ Primary cutaneous CD30-positive T-cell lymphoproliferative disorders.
▪ Primary cutaneous anaplastic large cell lymphoma.
▪ Lymphomatoid papulosis.
▪ Angioimmunoblastic T-cell lymphoma.
▪ Peripheral T-cell lymphoma, unspecified.
▪ Anaplastic large cell lymphoma.

T-cell lymphoma

A malignant proliferation of T cells arising in the skin, diagnosed by detecting rearrangement of the T-cell receptor's β chain; TCLs are often 'driven' by EBV and other viral infections; 90% of all Pts with TCL have extracutaneous involvement when diagnosed. See Mycosis fungoides. T-cell lymphomas Small lymphocytic (well-differentiated lymphocyte-like) lymphoma–13% Convoluted cell lymphoma or poorly-differentiated lymphocytic lymphoma–52% Immunoblastic sarcoma or 'histiocytic' lymphoma–19% Mycosis fungoides/Sézary syndrome–11% Lymphoepithelial cell (Lennert's) lymphoma–5% Cutaneous T-cell lymphoma HTLV (I, II)–induced lymphoma