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Blood, Vol. 95 No. 7 (April 1), 2000:
pp. 2212-2218
REVIEW ARTICLE
Transformation of mycosis fungoides: clinicopathological and
prognostic features of 45 cases
Béatrice Vergier,
Anne de Muret,
Marie Beylot-Barry,
Loïc Vaillant,
Didier Ekouevi,
Geneviève Chene,
Agnès Carlotti,
Nathalie Franck,
Pierre Dechelotte,
Pierre Souteyrand,
Philippe Courville,
Pascal Joly,
Michèle Delaunay,
Martine Bagot,
Florent Grange,
Sylvie Fraitag,
Jacques Bosq,
Tony Petrella,
Anne Durlach,
Antoine De Mascarel,
Jean-Philippe Merlio, and
Janine Wechsler for the French Study Group on Cutaneous
Lymphomas
From the Departments of Pathology, Dermatology, and Statistics,
University Hospital, Bordeaux, France; the Departments of Pathology and
Dermatology, University Hospital, Tours, France; the Departments of
Pathology and Dermatology, University Hospital, Tarnier, Paris V,
Paris, France; the Departments of Pathology and Dermatology, University
Hospital, Clermont Ferrand, France; the Departments of Pathology and
Dermatology, University Hospital, Rouen, France; the Departments of
Dermatology and Pathology, University Hospital, Creteil, Paris XII,
Paris, France; the Department of Dermatology, University Hospital,
Colmar, France; the Department of Pathology, University Hospital,
Necker, Paris V, Paris, France; the Department of Pathology, Gustave
Roussy Institute, Paris, France; the Department of Pathology,
University Hospital, Dijon, France; and the Department of Pathology,
University Hospital, Reims, France.
The course of mycosis fungoides (MF) is indolent except when
transformation to a large T-cell lymphoma occurs. The diagnosis of
transformed MF (T-MF) relies on the presence of more than 25% of large
cells on biopsy of an MF lesion. We analyzed 45 patients with T-MF
recorded by the French Study Group on Cutaneous Lymphomas to better
determine clinicopathological features of MF transformation and to
analyze their impact on prognosis. Median time from diagnosis of MF to
transformation was 6.5 years. Extracutaneous progression was present in
20 patients. Mean survival from transformation to death was 22 months.
In univariate analysis, only an extracutaneous progression was
associated with a worse prognosis (5-year actuarial survival: 7.8%
versus 32%). Neither sex, age, clinical and skin disease stage at
transformation, transformation speed, nor percentage of large
cells or CD30 expression (14 of 45) had a prognostic value. When
performing multivariate analysis, age (at least 60 years), and
extracutaneous spreading were found to be associated with a poor
prognosis. There was no difference between survival curves of patients
with T-MF and with pleomorphic large T-cell CD30 lymphomas. The main
diagnostic pitfall was "histiocytic-rich" MF, requiring CD68
staining for the diagnosis of T-MF. Out of 45 patients, 6 presented an
histologic transformation before clinical progression, suggesting that
an early histopathological diagnosis may be performed by histological
follow-up. The prognostic value of such early histopathological
diagnosis must be confirmed by prospective studies.
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