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- Title
Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides.
- Authors
van Santen, Suzanne; Roach, Rachel E. J.; van Doorn, Remco; Horváth, Barbara; Bruijn, Marjolein S.; Sanders, Cornelus J. G.; de Pooter, Jacco C.; van Rossum, Michelle M.; de Haas, Ellen R. M.; Veraart, Joep C. J. M.; Bekkenk, Marcel W.; Vermeer, Maarten H.; Willemze, Rein
- Abstract
<bold>Importance: </bold>Large case series suggest that patients with folliculotropic mycosis fungoides (FMF) have a worse prognosis than patients with classic mycosis fungoides (MF). However, recent studies described a subgroup of patients with FMF with a more favorable prognosis. Distinction between indolent and aggressive FMF may have important therapeutic consequences but is hampered by the inability of the current tumor-node-metastasis-blood (TNMB) staging system to classify patients with FMF in a clinically meaningful way.<bold>Objective: </bold>To differentiate between indolent and aggressive FMF using clinicopathological criteria and to define prognostic factors in patients with FMF.<bold>Design, Setting, and Participants: </bold>In this prospective cohort study, we followed 203 patients with FMF, included in the Dutch Cutaneous Lymphoma Registry between October 1985 and May 2014 at a tertiary referral center hosting the Dutch Cutaneous Lymphoma Registry. Overall, 220 patients with FMF had been registered, but 17 patients with incomplete follow-up data or a history of classic MF were excluded.<bold>Main Outcomes and Measures: </bold>Main outcomes included clinical and histological characteristics, disease progression, and survival. Prognostic factors were investigated using Cox proportional hazard regression analysis. Distinction between early plaque-stage FMF and advanced plaque-stage FMF was made by a blinded review of skin biopsy specimens from patients presenting with plaques.<bold>Results: </bold>In a cohort of 147 men and 56 women (median [range] age, 59 [15-93] years), patients with histologically early plaque-stage FMF had a very similar overall survival (OS) rate to patients with only patches and/or follicular papules (10-year OS, 71% vs 80%), while the survival rate of patients with histologically advanced plaque-stage FMF was almost identical to that of patients presenting with tumors (10-year OS, 25% vs 27%). Subsequently, 3 clinical subgroups with significantly different survival data were distinguished: early skin-limited FMF (group A; n = 84; 5-year and 10-year OS, 92% and 72%); advanced skin-limited FMF (group B; n = 102; 5-year and 10-year OS, 55% and 28%); and FMF presenting with extracutaneous disease (group C; n = 17; 5-year and 10-year OS, 23% and 2%). Age at diagnosis, large cell transformation and secondary bacterial infection were independent risk factors for disease progression and/or poor survival.<bold>Conclusions and Relevance: </bold>The results of this study provide useful criteria to differentiate between indolent and aggressive FMF and confirm the existence of a subgroup of FMF with a favorable prognosis.
- Subjects
BACTERIAL disease complications; HAIR follicles; HEAD tumors; LONGITUDINAL method; MYCOSIS fungoides; NECK tumors; PROGNOSIS; SCALP; SKIN tumors; SURVIVAL; TUMOR classification; DISEASE progression; DISEASE complications
- Publication
JAMA Dermatology, 2016, Vol 152, Issue 9, p992
- ISSN
2168-6068
- Publication type
journal article
- DOI
10.1001/jamadermatol.2016.1597