Novel agents are improving the outlook for patients with rare peripheral T cell lymphomas (PTCL) – offering better survival and improved tolerability than chemotherapy.

Speaking to the limbic about a new consensus statement on the diagnosis, management and follow-up of PTCL, Dr Dejan Radeski said the arrival of new therapies underpinned the need for a practice update.

He said outcomes in PTCL had been held back by variability in practice and had not yet achieved the kind of results seen in B cell lymphomas.

Dr Radeski, from the Sir Charles Gairdner Hospital in Perth, said the consensus statement brought together the latest evidence with clear recommendations across PTCL subtypes and treatment algorithms.

Given there were only about 500 new cases seen in Australia each year, Dr Radeski said there was some mystique about PTCL to break through.

“It’s all about giving clinicians around Australia a source document to help guide them through.”

He stressed presentation of cases to a multidisciplinary team meeting including an expert lymphoma pathologist was important to meet the challenge of diagnosis and classification.

Novel agents

While CHOP is the recommended first-line treatment for most subtypes, novel agents are starting to make their mark.

As reported in the limbic recently, the CD30 target therapy brentuximab is now PBS-listed for PTCL.

The brentuximab plus CHP regimen has also been shown to be superior to CHOP in anaplastic large cell lymphoma (ALCL).

However the highly mutated angioimmunoblastic T-cell lymphoma (AITL) benefit from other epigenetically targeted drugs such as hypomethylating agents and histone deacetylase inhibitors.

Dr Radeski predicted that in 10-15 years each subtype will have its own treatment algorithm.

Meanwhile, novel agents as second line therapy are providing better outcomes in patients ineligible for ASCT.

“We’ve seen two very good papers showing novel agents are superior to conventional chemotherapy in relapsed PTCL. If you can transplant, that’s great. But if you can’t, the novel agents give better outcomes,” he said.

Dr Radeski said endless lines of chemotherapy were not appropriate or effective.

The consensus statement also recommended repeat biopsy of relapsed/refractory disease prior to initiation of salvage therapy.

“We find that patients evolve e.g. from diagnosis when they don’t have CD30 expression to relapsed disease when they do.”

Dr Radeski said trials of new agents such as AFM13 were underway in relapsed/refractory CD30-positive T-cell lymphoma.

The consensus statement has been published in the Internal Medicine Journal.