Increased Risk of Subsequent Cancers in Patients With Sézary Syndrome - Summary - MDSpire
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Higher Risk of Subsequent Cancers Among Patients With Sézary Syndrome

  • By

  • Julia Nenonen

  • Despoina Tsakona

  • Philip Curman

  • Hanna Brauner

  • October 26, 2026

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Objective:

To assess subsequent cancer risk in adults with Sézary syndrome (SS) compared with matched comparators without mature T/NK-cell lymphomas.

Approach:
  • Design and data: Retrospective cohort analysis of US Collaborative Network data from TriNetX, covering August 2004 to August 2024.
  • Participants and matching: After propensity-score matching, the primary analysis included 2,031 adults with SS and 2,031 comparators. Matching included age, race, sex, nicotine-related factors, family cancer history, and overweight or obesity.
  • Outcomes and analysis: Subsequent primary malignancies were assessed over five years, with sensitivity analyses for 0–2 and 2–5 years. Hazard ratios were estimated using univariate Cox regression; no multiple-comparison adjustment was performed.
Key Findings:
  • Over five years, SS was associated with approximately sixfold higher risk of all cancers, twofold higher risk of solid cancers, 3.5-fold higher risk of skin cancers, and 50-fold higher risk of hematologic cancers than in matched comparators.
  • Follicular lymphoma had the highest hazard ratio among the reported outcomes.
  • Risks of melanoma, squamous cell carcinoma, and basal cell carcinoma were significantly elevated.
  • Sensitivity analyses for 0–2 and 2–5 years produced findings comparable to the overall five-year analysis.
  • In a comorbidity subanalysis, patients with SS generally had a lower comorbidity burden than matched comparators.
Interpretation:

The authors report that SS was associated with elevated risks across examined skin cancers, multiple hematologic malignancies, and aggregate cancer categories. They note that the study was exploratory and that the mechanisms underlying these associations were not established.

Limitations:
  • The study was exploratory because SS is rare and the cohort size was limited; no adjustment for multiple comparisons was performed.
  • The analysis used aggregated electronic health record data and ICD-10-CM codes; the supplied context does not describe additional limitations.
Sources:

Original Source(s)

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