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Cutaneous T-cell lymphoma.

P S Schein, J S Macdonald, R Edelson

    Cancer
    |October 1, 1976
    PubMed
    Summary
    This summary is machine-generated.

    Sézary Syndrome and mycosis fungoides are clinically indistinguishable, representing variant expressions of cutaneous T-cell lymphoma. Researchers propose a unified diagnostic term to replace archaic nomenclature.

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    Area of Science:

    • Dermatology
    • Oncology
    • Hematology

    Background:

    • Sézary Syndrome and mycosis fungoides are distinct clinical entities in current medical literature.
    • Both conditions involve the proliferation of malignant thymus-derived lymphocytes.

    Purpose of the Study:

    • To compare the clinical features and natural history of Sézary Syndrome and mycosis fungoides.
    • To evaluate the relationship between these two disorders and propose unified terminology.

    Main Methods:

    • Comparative analysis of detailed features and natural history.
    • Documentation of transitions between the two syndromes in 12 patients.

    Main Results:

    • Sézary Syndrome and mycosis fungoides are indistinguishable, except for the presence of a leukemic phase in Sézary Syndrome.

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  • Both disorders exhibit progressive involvement of skin, lymph nodes, and visceral organs.
  • Direct transitions between the two syndromes were observed.

    • Conclusions:

    • Sézary Syndrome and mycosis fungoides are variant clinical presentations of the same underlying disease process.
    • The current terminology is confusing and archaic.
    • A new designation, "cutaneous T-cell lymphoma, with or without leukemic phase," is recommended for all future descriptions.