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

MARVIN LUTZNER, M.D.; RICHARD EDELSON, M.D.; PHILIP SCHEIN, M.D., F.A.C.P.; IRA GREEN, M.D.; CHARLES KIRKPATRICK, M.D.; and AFTAB AHMED, Ph.D.
[+] Article and Author Information

▸Requests for reprints should be addressed to Marvin A. Lutzner, M.D., Chief, Dermatology Branch, National Cancer Institute, Bldg. 10, Room 12N-238, National Institutes of Health, Bethesda, MD 20014.


Bethesda, Maryland


Ann Intern Med. 1975;83(4):534-552. doi:10.7326/0003-4819-83-4-534
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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 lymphoproliterative 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.

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