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Blood, Vol. 93 No. 11 (June 1), 1999:
pp. 3637-3642
Prognostic Factors in Primary Cutaneous Lymphomas Other Than Mycosis
Fungoides and the Sézary Syndrome
F. Grange,
G. Hedelin,
P. Joly,
M. Beylot-Barry,
M. D'Incan,
M. Delaunay,
L. Vaillant,
M.F. Avril,
J. Bosq,
J. Wechsler,
S. Dalac,
C. Grosieux,
N. Franck,
E. Esteve,
C. Michel,
C. Bodemer,
B. Vergier,
L. Laroche, and
M. Bagot for the French Study Group on Cutaneous
Lymphomas (FSGCL)
From the Service de Dermatologie, Hôpital Pasteur, Colmar;
Département d'Epidémiologie et de Santé Publique,
Université Louis Pasteur, Strasbourg; Clinique Dermatologique,
Hôpital Charles Nicolle, Rouen, Institut National de la Sante et
de la Recherche Medicale (INSERM) Unite 519; Service de Dermatologie,
Hôpital du Haut Lévêque, Pessac; Service de
Dermatologie, Hôtel Dieu, Clermont-Ferrand; Unité de
Dermatologie-Cancérologie, Hôpital Pellegrin, Bordeaux;
Service de Dermatologie, Hôpital Trousseau, Tours; Service de
Dermatologie, Institut Gustave Roussy, Villejuif; Département
d'Histopathologie, Institut Gustave Roussy, Villejuif; Department de
Pathologie, Hôpital Henri-Mondor, Créteil; Service de
Dermatologie, Hôpital du Bocage, Dijon; Service de Dermatologie,
Hôpital Robert Debré, Reims; Service de Dermatologie,
Hôpital Tarnier, Paris; Service de Dermatologie, Hôpital
Porte Madeleine, Orléans; Service de Dermatologie,
Hôpital du Moenschberg, Mulhouse; Service de Dermatologie,
Hôpital Necker, Paris; Service d'Anatomie Pathologique,
Hôpital du Haut Lévêque, Pessac; Service de
Dermatologie, Hôpital Avicenne, Bobigny; and Service de
Dermatologie, Hôpital Henri-Mondor, Créteil, France.
Prognostic studies of primary cutaneous lymphomas (PCL) other than
mycosis fungoides (MF) and the Sézary syndrome (SS; non-MF/SS PCL) have been mainly performed on subgroups or on small numbers of
patients by using univariate analyses. Our aim was to identify independent prognostic factors in a large series of patients with non-MF/SS PCL. We evaluated 158 patients who were registered in the
French Study Group on Cutaneous Lymphomas database from January 1, 1986 to March 1, 1997. Variables analyzed for prognostic value were: age;
sex; type of clinical lesions; maximum diameter, location, and number
of skin lesions; cutaneous distribution (ie, local, regional, or
generalized); prognostic group according to the European Organization
for Research and Treatment of Cancer (EORTC) classification for PCL; B-
or T-cell phenotype; serum lactate dehydrogenase (LDH) level; and B
symptoms. Univariate and multivariate analyses were performed using a
model of relative survival. Forty-nine patients (31%) died. The median
relative survival time was 81 months. In univariate analysis, EORTC
prognostic group, serum LDH level, B symptoms, and variables related to
tumor extension (ie, distribution, maximum diameter, and number of skin
lesions) were significantly associated with survival. When these
variables were considered together in a multivariate analysis, EORTC
prognostic group and distribution of skin lesions remained
statistically significant, independent prognostic factors. This study
confirms the good predictive value of the EORTC classification for PCL
and shows that the distribution of skin lesions at initial evaluation
is an important prognostic indicator.
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