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New guidelines may miss some rare lymphoma cases



Intermediate magnification micrograph of cutaneous T-cell lymphoma, H&E stain, photo by: Nephron via Wikimedia Commons

A study published in the journal Blood raises concerns about current diagnostic guidelines for cutaneous T-cell lymphomas (CTCLs). Senior author Dr. Joan Guitart, chief of Dermatopathology at Northwestern Memorial Hospital and the Feinberg School of Medicine in Chicago, and colleagues report that recent updates to diagnostic criteria might lead to underdiagnosis of certain CTCL cases.

 

“At Northwestern, we have one of the largest cohorts of patients with cutaneous T-cell lymphomas,” said Dr. Guitart, who also leads the cutaneous lymphoma clinic at Northwestern Memorial Hospital. In a news release, he added: “We noted a few years ago that some patients who had the leukemic variant of cutaneous T-cell lymphoma were being underdiagnosed using recently updated diagnostic criteria. We suspected that it may have something to do with the modified criteria being used for diagnosis.”


CTCLs affect approximately 12 people per million annually. In this study, the researchers compared the 2007 and 2022 diagnostic guidelines by analyzing blood samples from 99 CTCL patients. They found that the newer criteria, which focus on total abnormal cell count rather than percentage, failed to identify nearly 10% of cases.


“About 10 per cent of the population were underdiagnosed or misdiagnosed with the new proposed criterion,” Dr. Guitart added. “That’s an important issue because we want to act quickly to treat our patients.”


The study noted that misdiagnosed patients typically presented with lymphopenia but faced similarly poor prognoses. Additionally, the new criteria were not able to accurately differentiate between disease stages, which is crucial information for predicting patient outcomes.


To address these concerns, Dr. Guitart and his colleagues suggest revising the blood staging criteria. “With this article, we are calling to the attention of clinicians that there is a flaw with the new criteria,” Dr. Guitart said. “We are proposing a revision of blood staging (B0, B1 and B2) criteria that more accurately identifies patients who are at risk of progression, often leading to a poor outcome.”


The authors are in discussions with the International Society of Cutaneous Lymphomas and other groups about potentially modifying the diagnostic criteria. They argue that improving staging criteria is necessary to ensure that patients with low tumour burden at presentation are not missed.


This study highlights the ongoing challenges in diagnosing and staging rare cancers such as CTCL. As diagnostic guidelines continue to evolve, feedback from clinicians and researchers remains crucial for refining approaches to patient care. The potential revisions suggested by this study could impact early detection and treatment strategies for CTCL patients, though the study authors agree that further research and consensus would be needed to implement any changes to current guidelines.


“Our findings provide an impetus to all the involved national and international societies to improve the staging criteria to find a way that does not miss the subset of patients with low tumour burden at presentation, which is what’s happening with the present updated criteria,” Dr. Guitart said.

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