
Peripheral T-Cell Lymphoma (PTL)
See NZLG (2014) T-cell non Hodgkin Lymphoma Protocol.
- This group of aggressive T-cell lymphomas probably includes a number of different entities. PTL has to be distinguished from angioimmunoblastic T-cell lymphoma, anaplastic large cell lymphoma, acute T-cell leukaemia/lymphoma and T-cell rich B-cell lymphoma.
- PTL may have nodal or extra-nodal disease often with bone marrow involvement and is an aggressive lymphoma that responds poorly to treatment.
- Immunophenotype CD2, 3, 4, 7 are usually positive, CD8- and CD30+. The TCR gene is clonally rearranged. No consistent cytogenetic changes have been observed although trisomy 3 is common in the Lennert variant.
Management
Intensive treatment (e.g. CHOP) may control the disease but long-term survival is poor (under 20%). Transplantation in first remission should be discussed in younger patients.
Further reading
Broccoli, A. and P.L. Zinzani (2017). "Peripheral T-cell lymphoma, not otherwise specified." Blood 129(9):1103.
Beaven, A.W.et al. (2015). "Peripheral T-cell lymphoma, NOS, and anaplastic large cell lymphoma." Hematology Am Soc Hematol Educ Program 2015: 550-8.
Topic Code: 5962