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Clinicopathologic Features and Long-Term Outcomes of Adult Hypopigmented Mycosis Fungoides

JAMA Dermatology·June 17Open Access
DermatologyConfirms priorCutaneous T-Cell LymphomaHypopigmented Mycosis FungoidesRetrospective Multicenter Cohort StudySkin-Directed TherapyAdult

Summary

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What was studied

This retrospective multicenter cohort study (6 US tertiary centers, Jan 2011–Oct 2023) examined clinicopathologic features and long-term outcomes in 224 adults (≥18 years) with biopsy-confirmed hypopigmented mycosis fungoides (HMF), with a median follow-up of 39 months.

Key findings

98% presented with early-stage disease; 77% achieved complete or partial response to treatment (26% CR, 51% PR), mostly with skin-directed therapy alone (90%). Only 6% progressed to a higher stage overall, and just 2% of early-stage patients advanced to advanced-stage disease. Peripheral blood TCR monoclonality (found in 27%) was linked to poorer treatment response but not to disease progression.

Study limitations

Retrospective design with inherent selection bias from tertiary referral centers; immunophenotyping and peripheral blood TCR data were unavailable for a substantial subset of patients (only 141/224 had immunophenotyping, only 85/224 had TCR testing); median follow-up of ~39 months may be insufficient to capture late progression events.

Clinical implications

Adult HMF, which disproportionately affects Black women, follows an indolent course and responds well to skin-directed therapy regardless of CD8 vs. CD4 predominance or mixed-variant presentation. Peripheral blood TCR monoclonality may signal lower treatment response and warrants closer monitoring, but should not alone escalate therapy.

Related Questions

Explore related topics

How does hypopigmented mycosis fungoides differ in presentation and prognosis between Black and White patients?What skin-directed therapies are most effective for early-stage mycosis fungoides?What is the significance of peripheral blood T-cell receptor monoclonality in cutaneous T-cell lymphoma?

Publication Details

Year
2026
Journal
JAMA Dermatology
Sample Size
n=224
Source
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