Histopathologic staging at initial diagnosis of mycosis fungoides and the Sezary syndrome. Definition of three distinctive prognostic groups

E. A. Sausville, J. L. Eddy, R. W. Makuch, A. B. Fischmann, G. P. Schechter, M. Matthews, E. Glatstein, D. C. Ihde, F. Kaye, S. R. Veach, R. Phelps, T. O'Connor, J. B. Trepel, J. D. Cotelingam, A. F. Gazdar, J. D. Minna, P. A. Bunn

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Abstract

Study Objective: To determine the optimal staging evaluation at the time of initial diagnosis of mycosis fungoides or the Sezary syndrome. Design: Retrospective review of a uniformly staged inception cohort. Setting: Single-institution tertiary care center. Patients: 152 consecutive patients who had mycosis fungoides with or without the Sezary syndrome within 6 months of the initial definitive diagnosis. Intervention: A detailed staging evaluation including physical examination, routine laboratory studies, chest roentgenogram, lymphangiogram, peripheral blood smear, lymph node biopsy, bone marrow aspirate or biopsy, and liver biopsy in selected patients. Measurements and Main Results: Univariate adverse prognostic features at initial diagnosis in patients with mycosis fungoides included (P < 0.01) one or more cutaneous tumors or generalized erythroderma, adenopathy, blood smear involvement with Sezary cells, lymph node effacement, eosinophilia, and visceral involvement. Important, independent prognostic factors in a multivariate analysis are the presence of visceral disease and type of skin involvement. Conclusions: A staging system based on histopathologic evaluation of skin, lymph nodes, blood, and visceral sites provides more comprehensive prognostic information than clinical evaluation of skin disease and adenopathy. Patients may be divided at initial presentation into three prognostic groups: good-risk patients, who have plaque-only skin disease without lymph node, blood, or visceral involvement (median survival, > 12 years); intermediate-risk patients, who have cutaneous tumors, erythroderma, or plaque disease with node or blood involvement but no visceral disease or node effacement (median survival, 5 years); and poor-risk patients, who have visceral involvement or node effacement (median survival, 2.5 years).

Original languageEnglish (US)
Pages (from-to)372-382
Number of pages11
JournalAnnals of Internal Medicine
Volume109
Issue number5
StatePublished - 1988

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Sezary Syndrome
Mycosis Fungoides
Biopsy
Exfoliative Dermatitis
Survival
Tertiary Care Centers
Physical Examination
Thorax
Lymph Nodes
Bone Marrow
Skin
Liver
Neoplasms

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Sausville, E. A., Eddy, J. L., Makuch, R. W., Fischmann, A. B., Schechter, G. P., Matthews, M., ... Bunn, P. A. (1988). Histopathologic staging at initial diagnosis of mycosis fungoides and the Sezary syndrome. Definition of three distinctive prognostic groups. Annals of Internal Medicine, 109(5), 372-382.

Histopathologic staging at initial diagnosis of mycosis fungoides and the Sezary syndrome. Definition of three distinctive prognostic groups. / Sausville, E. A.; Eddy, J. L.; Makuch, R. W.; Fischmann, A. B.; Schechter, G. P.; Matthews, M.; Glatstein, E.; Ihde, D. C.; Kaye, F.; Veach, S. R.; Phelps, R.; O'Connor, T.; Trepel, J. B.; Cotelingam, J. D.; Gazdar, A. F.; Minna, J. D.; Bunn, P. A.

In: Annals of Internal Medicine, Vol. 109, No. 5, 1988, p. 372-382.

Research output: Contribution to journalArticle

Sausville, EA, Eddy, JL, Makuch, RW, Fischmann, AB, Schechter, GP, Matthews, M, Glatstein, E, Ihde, DC, Kaye, F, Veach, SR, Phelps, R, O'Connor, T, Trepel, JB, Cotelingam, JD, Gazdar, AF, Minna, JD & Bunn, PA 1988, 'Histopathologic staging at initial diagnosis of mycosis fungoides and the Sezary syndrome. Definition of three distinctive prognostic groups', Annals of Internal Medicine, vol. 109, no. 5, pp. 372-382.
Sausville, E. A. ; Eddy, J. L. ; Makuch, R. W. ; Fischmann, A. B. ; Schechter, G. P. ; Matthews, M. ; Glatstein, E. ; Ihde, D. C. ; Kaye, F. ; Veach, S. R. ; Phelps, R. ; O'Connor, T. ; Trepel, J. B. ; Cotelingam, J. D. ; Gazdar, A. F. ; Minna, J. D. ; Bunn, P. A. / Histopathologic staging at initial diagnosis of mycosis fungoides and the Sezary syndrome. Definition of three distinctive prognostic groups. In: Annals of Internal Medicine. 1988 ; Vol. 109, No. 5. pp. 372-382.
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abstract = "Study Objective: To determine the optimal staging evaluation at the time of initial diagnosis of mycosis fungoides or the Sezary syndrome. Design: Retrospective review of a uniformly staged inception cohort. Setting: Single-institution tertiary care center. Patients: 152 consecutive patients who had mycosis fungoides with or without the Sezary syndrome within 6 months of the initial definitive diagnosis. Intervention: A detailed staging evaluation including physical examination, routine laboratory studies, chest roentgenogram, lymphangiogram, peripheral blood smear, lymph node biopsy, bone marrow aspirate or biopsy, and liver biopsy in selected patients. Measurements and Main Results: Univariate adverse prognostic features at initial diagnosis in patients with mycosis fungoides included (P < 0.01) one or more cutaneous tumors or generalized erythroderma, adenopathy, blood smear involvement with Sezary cells, lymph node effacement, eosinophilia, and visceral involvement. Important, independent prognostic factors in a multivariate analysis are the presence of visceral disease and type of skin involvement. Conclusions: A staging system based on histopathologic evaluation of skin, lymph nodes, blood, and visceral sites provides more comprehensive prognostic information than clinical evaluation of skin disease and adenopathy. Patients may be divided at initial presentation into three prognostic groups: good-risk patients, who have plaque-only skin disease without lymph node, blood, or visceral involvement (median survival, > 12 years); intermediate-risk patients, who have cutaneous tumors, erythroderma, or plaque disease with node or blood involvement but no visceral disease or node effacement (median survival, 5 years); and poor-risk patients, who have visceral involvement or node effacement (median survival, 2.5 years).",
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T1 - Histopathologic staging at initial diagnosis of mycosis fungoides and the Sezary syndrome. Definition of three distinctive prognostic groups

AU - Sausville, E. A.

AU - Eddy, J. L.

AU - Makuch, R. W.

AU - Fischmann, A. B.

AU - Schechter, G. P.

AU - Matthews, M.

AU - Glatstein, E.

AU - Ihde, D. C.

AU - Kaye, F.

AU - Veach, S. R.

AU - Phelps, R.

AU - O'Connor, T.

AU - Trepel, J. B.

AU - Cotelingam, J. D.

AU - Gazdar, A. F.

AU - Minna, J. D.

AU - Bunn, P. A.

PY - 1988

Y1 - 1988

N2 - Study Objective: To determine the optimal staging evaluation at the time of initial diagnosis of mycosis fungoides or the Sezary syndrome. Design: Retrospective review of a uniformly staged inception cohort. Setting: Single-institution tertiary care center. Patients: 152 consecutive patients who had mycosis fungoides with or without the Sezary syndrome within 6 months of the initial definitive diagnosis. Intervention: A detailed staging evaluation including physical examination, routine laboratory studies, chest roentgenogram, lymphangiogram, peripheral blood smear, lymph node biopsy, bone marrow aspirate or biopsy, and liver biopsy in selected patients. Measurements and Main Results: Univariate adverse prognostic features at initial diagnosis in patients with mycosis fungoides included (P < 0.01) one or more cutaneous tumors or generalized erythroderma, adenopathy, blood smear involvement with Sezary cells, lymph node effacement, eosinophilia, and visceral involvement. Important, independent prognostic factors in a multivariate analysis are the presence of visceral disease and type of skin involvement. Conclusions: A staging system based on histopathologic evaluation of skin, lymph nodes, blood, and visceral sites provides more comprehensive prognostic information than clinical evaluation of skin disease and adenopathy. Patients may be divided at initial presentation into three prognostic groups: good-risk patients, who have plaque-only skin disease without lymph node, blood, or visceral involvement (median survival, > 12 years); intermediate-risk patients, who have cutaneous tumors, erythroderma, or plaque disease with node or blood involvement but no visceral disease or node effacement (median survival, 5 years); and poor-risk patients, who have visceral involvement or node effacement (median survival, 2.5 years).

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