Image Description
Most PTCL, NOS show TCR gene rearrangements. Clonality is detected in more than 90% of cases when both TCRβ and TCRγ are tested. Immunoglobulin heavy-chain gene rearrangements are noted in about a third of cases, usually associated with the presence of EBV+ B-cells or evidence of B-cell expansion. With cytogenetics, karyotypic abnormalities are detected in virtually all cases. Complex karyotypes are seen with large cell morphology and are linked to poor outcomes. Trisomy 3 is frequently seen in the lymphoepithelioid variant. The rare follicular variant of PTCL, NOS shows recurrent translocation t(5;9)(q33;q22). Based on the gene expression studies, PTCL, NOS are found to be a heterogenous group with different subgroups having different molecular profiles. These profiles may have prognostic relevance.
About the Disease
Peripheral T-cell lymphomas (PTCL) account for about 10% to 15% of all non-Hodgkin lymphomas in the United States. PTCLs are classified into various subtypes. Peripheral T-cell Lymphoma, Not Otherwise Specified (PTCL, NOS) is the most common subtype in North America and Europe where it comprises between 30% and 50% of all PTCLs. It is less common in Asia. It affects older individuals with the median age around 60 years. There is slight male predominance.Most patients present at advanced stages with nodal as well as extranodal involvement (bone marrow, liver, spleen, and skin). The clinical presentation includes generalized lymphadenopathy, sometimes accompanied by fever, eosinophilia, pruritus and weight loss.PTCL, NOS, are aggressive tumors that respond poorly to therapy and frequently relapse. The 5-yr overall survival rate is about 30%.