Cutaneous T-Cell Lymphomas: The Sézary Syndrome, Mycosis Fungoides, and Related Disorders

Abstract

Substantial evidence has accumulated to indicate not only that mycosis fungoides and the Sézary syndrome are closely related malignancies, but to suggest that they are part of a larger spectrum of cutaneous lymphomas. The neoplastic cells of these disorders have membrane features of thymus-derived (T) lymphocytes, a characteristic tissue distribution (skin infiltration, marrow sparing, localization in T-cell regions of lymphoid tissue), and distinctive morphology. For these reasons, we suggest that these lymphoproliferative disorders be grouped together as "cutaneous T-cell lymphomas". The anergy noted in patients of this group with leukemia probably is related to both decreased percentages of normal T cells and presence in the serum of macrophage migration inhibitory activity. Leukapheresis has been particularly effective in the management of selected patients. The homogeneous T-cell populations in the patients with leukemia also provide important opportunities to study many aspects of lymphocyte physiology that are of broad biologic significance.

Walter Hubert Lecture, delivered 1 April 1980 to the 21st AGM of the BACR Malignant lymphomas as tumours of the immune system

“…While uncommon in the United States, such tumours appear to have a greater frequency in Japan (Uchiyama et al, 1977;Suchi et al, 1979 (Jaffe et al, 1975;Waldron et al, 1977). Mycosis fungoides and Sezary's syndrome, although not included in most classifications of nonHodgkin's lymphomas, are also peripheral T-lymphocyte malignancies (Brouet et al, 1973;Flandrin & Brouet, 1974;Lutzner et al, 1975;Robinowitz et al, 1976). …”

The History of Lymphoma Classifications with Special Consideration of Cutaneous Lymphomas

“…Before 1965, lymphomatoid papulosis was known as "regressive atypical histiocytosis," "atypical paradoxical reticulopathy" cutaneous Hodgkin's disease, mycosis fungoides or as T-cell pseudolymphoma [75]. In 1975, based on morphological and immunological studies, Lutzner et al [103] mentioned the relation of LyP to the CTCL.…”

Mycosis Fungoides

“…Lutzner et al have questioned the na ture of some of the cases reported as chronic lymphocytic leukemia with an erythroderma. 4 They propose that erythroderma is a manifestation of T lymphocytes that preferentially reside in the'dermis.434…”

“…37 The concept of primary cutaneous T-cell dysplasia, leukemia and lymphoma in cludes disorders classified clinically as mycosis fungoides, parapsoriasis, poi kiloderma atrophicans and Sézary syn drome. 4,8 In each of these disorders, erythematous or atrophic lesions, whose patterns dominate much of the clinical life history, may give way to indurated lesions or tumors. 4 These alterations in basic patterns are clinical markers of progressive disease.…”

“…4,8 In each of these disorders, erythematous or atrophic lesions, whose patterns dominate much of the clinical life history, may give way to indurated lesions or tumors. 4 These alterations in basic patterns are clinical markers of progressive disease. 3 Correlates for the clinical markers are found in cytologic and histologic alter ations of the lymphoid infiltrates (his tologic progression).7 Histologic pro gression is manifested by increasing degrees of cytologic atypism and by an increasing proportion of atypical cells at the expense-of reactive cells.…”