Abstract
Background
Survival analysis of a large series of patients with mycosis fungoides (MF) and Sézary
syndrome (SS) has not been performed in Japan. Revision to the staging system for
MF and SS was recently published.
Objective
To determine the long-term prognosis of Japanese patients with MF and SS, to identify
factors predictive of survival, and to evaluate the prognostic significance of the
revised staging system.
Methods
We performed a retrospective cohort study of 100 Japanese patients with MF and SS
managed at the dermatology division of Niigata University Hospital between April 1,
1982 and March 31, 2006. We estimated survival according to the patient's clinical
characteristics including the stages, and assessed their prognostic significance.
Results
Survival rates of Japanese patients with MF and SS were similar to those shown in
previous studies conducted in the United States and Europe. Prognosis of patients
with skin tumor (stage IIB) and extracutaneous involvement (stage IV) was significantly
worse than that of those with early-stage disease (stages IA–IIA), but erythrodermic
MF patients without blood involvement (stage IIIA) showed excellent survival. Independent
prognostic factors in multivariate analyses were higher age and the presence of skin
tumor and extracutaneous disease.
Conclusion
Patient age and stage are the most important clinical prognostic factors in Japanese
patients with MF and SS. The revised staging system is useful for predicting survival
of the patients, but at least a subpopulation of stage IIIA patients may have a favorable
prognosis.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of Dermatological ScienceAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Mycosis fungoides.in: Swerdlow S.H. Campo E. Harris N.L. Jaffe E.S. Pileri S.A. Stein H. Thiele J. Vardiman J.W. WHO classification of tumours of haematopoietic and lymphoid tissues. IARC, Lyon2008: 296-298
- Sézary syndrome.in: Swerdlow S.H. Campo E. Harris N.L. Jaffe E.S. Pileri S.A. Stein H. Thiele J. Vardiman J.W. WHO classification of tumours of haematopoietic and lymphoid tissues. IARC, Lyon2008 (p. 299)
- Prognostic variables in mycosis fungoides.Cancer. 1981; 47: 2671-2677
- Clinical staging for cutaneous T-cell lymphoma.Ann Intern Med. 1984; 100: 187-192
- Histologic assessment of lymph nodes in mycosis fungoides/Sézary syndrome (cutaneous T-cell lymphoma): clinical correlations and prognostic import of a new classification system.Hum Pathol. 1985; 16: 1098-1109
- Evaluation of circulating malignant cells provides prognostic information in cutaneous T cell lymphoma.Blood. 1987; 69: 841-849
- Histopathologic staging at initial diagnosis of mycosis fungoides and the Sézary syndrome. Definition of three distinctive prognostic groups.Ann Intern Med. 1988; 109: 372-382
- Extracutaneous mycosis fungoides.Cancer. 1987; 60: 397-402
- Prognostic clinicopathologic factors in cutaneous T-cell lymphoma.Arch Dermatol. 1991; 127: 1511-1516
- Prognostic factors in erythrodermic mycosis fungoides and the Sézary syndrome.Arch Dermatol. 1995; 131: 1003-1008
- Long-term outcome of 525 patients with mycosis fungoides and Sézary syndrome: clinical prognostic factors and risk for disease progression.Arch Dermatol. 2003; 139: 857-866
- CD8-positive tumor-infiltrating lymphocytes influence the long-term survival of patients with mycosis fungoides.J Am Acad Dermatol. 1995; 32: 448-453
- Increased serum concentration of the soluble interleukin-2 receptor in cutaneous T-cell lymphoma: clinical and prognostic implications.Arch Dermatol. 1996; 132: 42-47
- Prognostic factors and evaluation of mycosis fungoides and Sézary syndrome.J Am Acad Dermatol. 1997; 37: 58-67
- The prognostic significance of delayed hypersensitivity to dinitrochlorobenzene and mechlorethamine hydrochloride in cutaneous T cell lymphoma.J Invest Dermatol. 1998; 110: 946-950
- Sézary cell counts in erythrodermic cutaneous T-cell lymphoma: implications for prognosis and staging.Leuk Lymphoma. 2006; 47: 1841-1856
- Prognostic factor analysis in mycosis fungoides/Sézary syndrome.J Am Acad Dermatol. 1999; 40: 914-924
- Prognosis in cutaneous T-cell lymphoma by skin stage: long-term survival in 489 patients.J Am Acad Dermatol. 1999; 40: 418-425
- The changing survival of patients with mycosis fungoides: a population-based assessment of trends in the United States.Cancer. 1999; 85: 208-212
- Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients.Arch Dermatol. 2000; 136: 504-510
- Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases. French Study Group of Cutaneous Lymphomas.Blood. 2000; 95: 2212-2218
- Detection of a peripheral blood T cell clone is an independent prognostic marker in mycosis fungoides.J Invest Dermatol. 2000; 114: 117-121
- Molecular staging of lymph nodes from 60 patients with mycosis fungoides and Sézary syndrome: correlation with histopathology and outcome suggests prognostic relevance in mycosis fungoides.Br J Dermatol. 2006; 155: 756-762
- Prognostic significance of tumor burden in the blood of patients with erythrodermic primary cutaneous T-cell lymphoma.Blood. 2001; 97: 624-630
- Prognostic factors in Sézary syndrome: a study of 28 patients.Br J Dermatol. 2003; 149: 1152-1158
- Prognostic factors and prediction of prognosis by the CTCL Severity Index in mycosis fungoides and Sézary syndrome.Br J Dermatol. 2005; 153: 118-124
- Analysis of T-cell receptor gene rearrangement for predicting clinical outcome in patients with cutaneous T-cell lymphoma: a comparison of Southern blot and polymerase chain reaction methods.Arch Dermatol. 2005; 141: 1107-1113
- Circulating CD8+ lymphocytes, white blood cells, and survival in patients with mycosis fungoides.Br J Dermatol. 2005; 153: 324-330
- Survival of mycosis fungoides in patients in the Southeast of England.Dermatology. 2005; 211: 325-329
- Overall survival in erythrodermic cutaneous T-cell lymphoma: an analysis of prognostic factors in a cohort of patients with erythrodermic cutaneous T-cell lymphoma.Int J Dermatol. 2009; 48: 243-252
- Report of the committee on staging and classification of cutaneous T-cell lymphomas.Cancer Treat Rep. 1979; 63: 725-728
- Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC).Blood. 2007; 110: 1713-1722
- Poor prognosis in non-Caucasian patients with early-onset mycosis fungoides.J Am Acad Dermatol. 2009; 60: 231-235
- Defining early mycosis fungoides.J Am Acad Dermatol. 2005; 53: 1053-1063
- Toxicity and response criteria of the Eastern Cooperative Oncology Group.Am J Clin Oncol. 1982; 5: 649-655
- Nonparametric estimation from incomplete observations.J Am Stat Assoc. 1958; 53: 457-481
- Evaluation of survival data and two new rank order statistics arising in its consideration.Cancer Chemother Rep. 1966; 50: 163-170
- Regression models and life tables.J R Stat Soc (B). 1972; 34: 187-220
- Adjusted p-values for simultaneous inferences.Biometrics. 1992; 48: 1005-1013
- A simple sequentially rejective multiple test procedure.Scand J Stat. 1979; 6: 65-70
- Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas.J Am Acad Dermatol. 2002; 46: 95-106
- Sézary syndrome and related variants of classic cutaneous T-cell lymphoma: a descriptive and prognostic clinicopathologic study of 29 cases.Leuk Lymphoma. 2003; 44: 59-69
- Tumor burden index as a prognostic tool for cutaneous T-cell lymphoma: a new concept.Arch Dermatol. 1999; 135: 1204-1208
- Clinical characteristics and long-term outcome of patients with generalized patch and/or plaque (T2) mycosis fungoides.Arch Dermatol. 1999; 135: 26-32
- Prognostic factors in Sézary syndrome: a multivariate analysis of clinical, haematological and immunological features.Ann Oncol. 1998; 9: 857-863
- Use of serum soluble interleukin-2 receptor levels to monitor the progression of cutaneous T-cell lymphoma.J Am Acad Dermatol. 1998; 38: 207-220
- Serological immunomarkers in cutaneous T cell lymphoma.Dermatology. 2004; 209: 296-300
- A predictive model for aggressive non-Hodgkin's lymphoma: the International Non-Hodgkin's Lymphoma Prognostic Factors Project.N Engl J Med. 1993; 329: 987-994
- Clinical stage IA (limited patch and plaque) mycosis fungoides: a long-term outcome analysis.Arch Dermatol. 1996; 132: 1309-1313
- Calculation of survival rates for cancer.Proc Staff Meet Mayo Clin. 1950; 25: 270-286
- Cancer survival analysis.in: Green F.L. Page D.L. Fleming I.D. Fritz A.G. Balch C.M. Haller D.G. Morrow M. AJCC cancer staging manual. 6th ed. Springer-Verlag, New York2002: 9-15
- Clinical characteristics and outcome of patients with extracutaneous mycosis fungoides.J Clin Oncol. 2001; 19: 779-784
Article info
Publication history
Published online: July 12, 2010
Accepted:
October 18,
2009
Received in revised form:
September 25,
2009
Received:
July 13,
2009
Identification
Copyright
© 2009 Japanese Society for Investigative Dermatology. Published by Elsevier Inc. All rights reserved.
