Abstract
In a 5-year interval (1984 to 1989), 32 patients with erythroderma caused by cutaneous T-cell lymphoma (CTCL) were evaluated in the Cutaneous Lymphoma Center at Hahnemann University. These patients were classified further as erythrodermic mycosis fungoides (E-MF) or Sézary syndrome (SS) on the basis of quantitative counts of Sézary cells on peripheral blood smears, and the two groups were compared using multiple clinical, histopathologic, and immuno-pathologic findings obtained at the time of presentation. Apart from differences related to leukemic involvement, statistically significant differences between the two subgroups of erythrodermic CTCL were found for the frequency of (i) documented lymph node involvement, (ii) a marked degree of tumor cell pleomorphism in skin biopsy specimens, and (iii) complete response to treatment lasting more than 6 months. The prognostic importance of these clinical and pathologic parameters was also determined by univariate analysis using the entire data set. The individual variables identified as possibly being associated with a favorable prognosis included (i) younger age of the patient, (ii) long duration of disease prior to study, (iii) 15 or fewer Sézary cells per 100 lymphocytes [the definition of the E-MF clinical subtype], (iv) presence of epidermal edema, (v) a dermal infiltrate composed predominantly of small lymphocytes, (vi and vii) absence of CD71 and CD30 positive tumor cells in the skin, and (viii) complete response to treatment. These variables were then entered into a Cox model for multivariate analysis. The patient’s age, clinical subtype of erythrodermic CTCL, presence or absence of tumor cells expressing the transferrin receptor (CD71, T9) in the skin, and response to treatment were the variables that correlated best with survival. The results of this study suggest that the differences between E-MF and SS are related more to degree of tumor burden than to etiopathogenesis. In addition, demonstration of blood involvement by quantitative Sézary cell counts is useful to define at least two prognostic groups within the erythrodermic CTCL spectrum and should be adopted for staging purposes.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Bakels V, JW van Oostveen, RLJ Gordijn, JMM Walboomers, CJLM Meijer, and R Willemze, 1991. Diagnostic value of T-cell receptor beta gene rearrangement analysis on peripheral blood lymphocytes of patients with erythroderma. J. Invest. Dermatol., 97: 782.
Buechner SA and RK Winkelmann, 1983. Pre-Sézary erythroderma evolving to Sézary syndrome. Arch. Dermatol., 119: 285.
Buechner SA and RK Winkelmann, 1983. Sézary syndrome. Arch. Dermatol., 119: 979.
Bunn PA Jr. and SI Lamberg, 1979. Report of the committee on staging and classification of cutaneous T-cell lymphomas. Cancer Treat. Rep., 63: 725.
Chu AC, D Robinson, JLM Hawk, R Meachum, NF Spittle and NP Smith, 1986. Immunologic differentiation of the Sézary syndrome due to cutaneous T-cell lymphoma and chronic actinic dermatitis. J. Invest. Dermatol., 86: 134.
Dmitrovsky E, MJ Matthews, PA Bunn, GP Schechter, RW Makuch, CF Winkler, J Eddy, EA Sausville and DC Ihde, 1987. Cytologic transformation in cutaneous T cell lymphoma: A clinicopathologic entity associated with poor prognosis. J. Clin. Oncol., 5: 208.
Imai S, G Burg and O Braun Falco, 1986. Mycosis fungoides and Sézary’s syndrome show distinct histomorphological features. Dermatologica, 173: 131.
Johnson SC, DJ Cripps and DH Norback, 1979. Actinic reticuloid: A clinical, pathologic and action spectrum study. Arch. Dermatol., 115: 1078.
Lessin SR, AH Rook and G Govera, 1991. Molecular diagnois of cutaneous T-cell lymphoma: Polymerase chain reaction amplification of T-cell antigen receptor beta-chain gene rearrangements. J. Invest. dermatol., 96: 299.
Lutzner M, R Edelson, P Schein, I Green, C Kirkpatrick and A Ahmed, 1975. Cutaneous T-cell lymphomas: The Sézary syndrome, mycosis fungoides and related disorders. Ann. Intern. Med., 83: S34.
Matutes E, D Robinson, M O’Brien, BF Haynes, H Zola, and D Catovsky, 1983. Candidate counterparts of Sézary cells and adult T-cell lymphoma-leukemia cells in normal peripheral blood: An ultrastructural study with the immunogold method and monoclonal antibodies. Leuk. Res., 7: 787.
Nasu K, J Said, EC Vonderheid, J Olerud, D Sako and M Kadin, 1985. Immunopathology of cutaneous T-cell lymphomas. Am. J. Pathol., 119: 436.
Neild VS, JLM Hawk, RAJ Eady and JJ Cream, 1982. Actinic reticuloid with Sézary cells. Clin. Exp. Dermatol., 7: 143.
Nowell PC, JB Finan and EC Vonderheid, 1982. Clonal characteristics of cutaneous T-cell lymphomas: Cytogenetic evidence from blood, lymph nodes and skin. J. Invest. Dermatol., 78: 69.
Rook AH, MB Prystowsky, M Cassin, M Boufal and SR Lessin, 1991. Combined therapy for Sézary syndrome with extracorporeal photochemotherapy with low-dose interferon alfa therapy. Arch. Dermatol., 127: 1535.
Rosenthal CJ, CA Noguera, A Coppola and SN Kapelner, 1982. Pseudolymphoma with mycosis fungoides manifestations, hyperresponsiveness to diphenylhydantoin, and lymphocyte disregulation. Cancer, 49: 2305.
Salhany KE, JB Cousar, JP Greer, TT Casey, JP Fields and RD Collins, 1988. Transformation of cutaneous T cell lymphoma to large cell lymphoma. Am. J. Pathol., 132: 265.
Schechter GP, EA Sausville, AB Fischmann, F Soehnlen, E Joyce, M Matthews, AF Gazdar, J Guccione, D Munson, R Makuch and PA Bunn, Jr, 1987. Evaluation of circulating malignant cells provides prognostic information in cutaneous T cell lymphoma. Blood, 69: 841.
Scheffer E, CJLM Meijer, WA van Vloten and R Willemze, 1986. A histologic study of lymph nodes from patients with the Sézary syndrome. Cancer, 57: 2375.
Schein PS, JS MacDonald and R Edelson, 1975. Cutaneous T-cell lymphoma. Cancer, 38: 1859.
Sentis HJ, R Willemze and E Scheffer, 1986. Histopathologic studies in Sézary syndrome and erythrodermic mycosis fungoides. A comparison with benign forms of erythroderma. J. Am. Acad. Dermatol., 15: 1217.
Toonstra J, H van Weelden, FHJ Gmelig Meyling, SC van der Putte, SI Schiere and H Bart de la Faille, 1985. Actinic reticuloid simulating Sézary syndrome: Report of two cases. Arch. Dermatol. Res., 277: 149.
Volkenandt M, HP Soyer, H Kerl and JR Bertino, 1991. Development of a highly specific and sensitive molecular probe for detection of cutaneous lymphoma. J. Invest. Dermatol., 97: 137.
Vonderheid EC, LW Diamond, S-M Lai, F Au, and MA DellaVecchia, 1992. Lymph node histopathology in cutaneous T-cell lymphoma: A prognostic classification system based on morphologic assessment. Am. J. Clin. Pathol., 97: 121.
Vonderheid EC, EL Sobel, PC Nowell, J Finan, M Helfrich and D Whipple, 1985. Diagnostic and prognostic significance for Sézary cells in peripheral blood smears from patients with cutaneous T-cell lymphoma. Blood, 66: 358.
Weinstock MA and JW Horm, 1988. Population-based estimate of survival and determinants of prognosis in patients with mycosis fungoides. Cancer, 62: 1658.
Weiss LM, GS Wood, E Hu, EA Abel, RT Hoppe and J Sklar, 1989. Detection of clonal T-cell receptor gene rearrangements in the peripheral blood of patients with mycosis fungoides/Sézary syndrome. J. Invest. Dermatol., 92: 601.
Wieselthier JS and HK Koh, 1990. Sézary syndrome: Diagnosis, prognosis, and critical review of treatment options. J. Am. Acad. Dermatol., 22: 381.
Willemze R, WA van Vloten, J Hermans, MJM Damsteeg and CJLM Meijer, 1983. Diagnostic criteria in Sézary’s syndrome: A multiparameter study of peripheral lymphocytes in 32 patients with erythroderma. J. Invest. Dermatol., 81: 392.
Winkelmann RK, SA Ruechner and JL Diaz Perez, 1984. Pre-Sézary syndrome. J. Am. Acad. Dermatol., 10: 992.
Winkelmann RK and JW Linman, 1973. Erythroderma with atypical lymphocytes (Sézary syndrome). Am. J. Med., 55: 192.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1994 Springer Science+Business Media New York
About this chapter
Cite this chapter
Vonderheid, E.C. et al. (1994). Analysis of Clinical, Histopathologic and Immunopathologic Parameters in Erythrodermic Variants of Cutaneous T-Cell Lymphoma with Implications for Staging. In: Lambert, W.C., Giannotti, B., van Vloten, W.A. (eds) Basic Mechanisms of Physiologic and Aberrant Lymphoproliferation in the Skin. NATO ASI Series, vol 265. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-1861-7_21
Download citation
DOI: https://doi.org/10.1007/978-1-4615-1861-7_21
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4613-5756-8
Online ISBN: 978-1-4615-1861-7
eBook Packages: Springer Book Archive