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Titolo:
PROGNOSTIC FACTORS IN SEZARY-SYNDROME - A MULTIVARIATE-ANALYSIS OF CLINICAL, HEMATOLOGICAL AND IMMUNOLOGICAL FEATURES
Autore:
BERNENGO MG; QUAGLINO P; NOVELLI M; CAPPELLO N; DOVEIL GC; LISA E; DEMATTEIS A; FIERRO MT; APPINO A;
Indirizzi:
UNIV TURIN,SECT DERMATOL 1,DEPT MED & SURG SPECIALTIES,VIA CHERASCO 23 I-10126 TURIN ITALY UNIV TURIN,SECT MED STAT,DEPT GENET BIOL & MED CHEM TURIN ITALY
Titolo Testata:
Annals of oncology
fascicolo: 8, volume: 9, anno: 1998,
pagine: 857 - 863
SICI:
0923-7534(1998)9:8<857:PFIS-A>2.0.ZU;2-D
Fonte:
ISI
Lingua:
ENG
Soggetto:
T-CELL LYMPHOMA; MYCOSIS-FUNGOIDES; GENE REARRANGEMENTS; CYTOKINE PROFILE; SURVIVAL; BLOOD; INTERLEUKIN-7; MARKER;
Keywords:
CUTANEOUS T-CELL LYMPHOMA; MULTIVARIATE ANALYSIS; SEZARY SYNDROME CLASSIFICATION; SEZARY SYNDROME IMMUNOLOGY; SEZARY SYNDROME PROGNOSIS; SEZARY SYNDROME THERAPY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
49
Recensione:
Indirizzi per estratti:
Citazione:
M.G. Bernengo et al., "PROGNOSTIC FACTORS IN SEZARY-SYNDROME - A MULTIVARIATE-ANALYSIS OF CLINICAL, HEMATOLOGICAL AND IMMUNOLOGICAL FEATURES", Annals of oncology, 9(8), 1998, pp. 857-863

Abstract

Background: Sezary syndrome (SS) prognostic factors are not well defined because of the rarity of this disease. The specific goal of this prospective study was to assess by multivariate analysis the predictivevalue with respect to survival of a series of clinical, haematological and immunological parameters taken at SS diagnosis. Patients and methods: A cohort of 62 SS patients diagnosed and followed since 1975 wasexamined, and 51 were included in the multivariate analysis model. Results: The median survival time was 31 months (range: 1 month-15.7+ years), and the five-year survival rate 33.5%. The following variables were found by univariate analysis to be associated with a poor prognosis at the time of SS diagnosis: previous history of mycosis fungoides (P = 0.013), high number of circulating leukocytes (P = 0.001), Sezary cells (SC) (P < 0.001) and CD4+ cells (P < 0.001), presence of large circulating SC (P < 0.001), above normal range LDH serum levels (P = 0.015), presence of PAS-positive inclusions in the cytoplasm of circulating SC (P < 0.001), high CD4/CD8 ratio (P = 0.004) and a CD7 negative circulating SC phenotype (P < 0.001). Among them, the stepwise multivariate analysis selected as adverse independent prognostic factors: PAS-positive cytoplasmic inclusions (P = 0.001), CD7 negative phenotype (P = 0.018) and presence of large circulating SC (P = 0.045). Conclusions: Two low-/high-risk groups have been singled out on the basis of the risk index. Patients with no or one adverse prognostic feature(s) (risk index less than or equal to 1; n = 31) share a slow disease courseand a relatively favorable prognosis (five-year survival. 58%); on the other hand, patients with 2 or 3 adverse prognostic features (risk index > 1; n = 20) are characterized by an aggressive disease course not modifiable by traditional therapies (five-year survival: 5%).

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Documento generato il 29/11/20 alle ore 16:01:31