LONG BEACH, CA – Among hematological malignancies, T-cell lymphomas have an extremely poor prognosis. A new review suggested that might be changing with unusual paths to cure.

An article in the Lancet Haematology pointed out that, over the last three decades, little progress has been made in changing the natural history of peripheral T-cell lymphomas.1

Long Beach, CA, VA Healthcare System-let researchers noted that one reason for the very poor prognosis for T-cell lymphomas is that no treatment program has ever been developed specifically for the broader category of the disease-peripheral T-cell lymphoma or any of the specific subtypes. The only exception, they added, has been advances made for patients with CD30-positive anaplastic large cell lymphoma.

Other authors were from the University of Virginia Cancer Center in Charlottesville.

“Decades of effort have focused on retrofitting chemotherapy programmes used for other diseases, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) for diffuse large B-cell lymphoma, which have not been associated with much progress, and have universally produced far more toxicity than benefit,” the authors wrote. “A remarkable heterogeneity, a paucity of cases, and the absence of peripheral T-cell lymphoma-specific drugs, until recently at least, have limited the field’s ability to make substantive and innovative advances.”

That appears to be changing, according to the report.

“Over the past few years, however, it appears the field is beginning to make progress,” according to study authors. “Lineage and disease-specific novel-to-novel platforms are producing, although perhaps not unsurprisingly, compelling results suggesting that the path to a cure for this rare orphan disease might be heading in a different direction.”

Another recent study discussed a subtype of T-cell lymphomas, cutaneous (CTCL), which  are a heterogeneous group of extranodal non-Hodgkin lymphomas. The report in Diagnostic Pathology pointed out that diagnosis can be challenging given the potential for overlap with inflammatory dermatoses.2

“Current diagnostic criteria for CTCL incorporate clinical and histopathologic findings as well as results of T-cell receptor (TCR) gene sequencing,” advised researchers from Stanford Medicine and the VA Palo Alto, CA, Healthcare System. “Molecular interrogation of TCR genes, TRG and TRB, has proven to be a critical tool for confirming diagnoses of CTCL and for disease tracking after initiation of therapy or after stem cell transplant. Methods for confirming a diagnosis of lymphoma in the absence of TCR gene clonality are lacking.”

The authors presented to case studies of patients with CTCL with pathogenic somatic mutations in the absence of TRG and TRB clonality, concluding, “These cases highlight how detection of pathogenic somatic mutations can confirm a diagnosis of lymphoma in a clinically and histopathologically suspicious cutaneous lymphoid proliferation without detectable TCR clonality.”

  1. Ma H, Marchi E, O’Connor OA. The peripheral T-cell lymphomas: an unusual path to cure. Lancet Haematol. 2020 Oct;7(10):e765-e771. doi: 10.1016/S2352-3026(20)30207-6. PMID: 32976753.
  2. Rojansky R, Fernandez-Pol S, Wang E, Rieger KE, et. Al .Cutaneous T-cell lymphomas with pathogenic somatic mutations and absence of detectable clonal T-cell receptor gene rearrangement: two case reports. Diagn Pathol. 2020 Sep 28;15(1):122. doi: 10.1186/s13000-020-01022-x. PMID: 32988392; PMCID: PMC7523289.