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What is Mycosis Fungoides? A Modest Proposal

  • W. Clark Lambert
Part of the NATO ASI Series book series (NSSA, volume 265)

Abstract

Mycosis fungoides, and related disorders such as Sézary syndrome, which comprise the diseases known collectively as “Cutaneous T Cell Lymphomas” (CTCL), are a heterogeneous group of disorders. Most of these patients experience insidious onset of a cutaneous eruption which shows features difficult or impossible to distinguish from those of a number of inflammatory dermatosis. During a variable, but usually prolonged, course, a cell type appears in the epidermis with a striking morphology, the so-called Sézary or Lutzner cell, depending on its size. In the leukemic forms of these diseases, the Sézary syndrome, these cells are also found in number in the peripheral blood. With further progression, these distinctive cells are found in nests within the epidermis, at which time a definitive diagnosis of mycosis fungoides may be made. These cells have cell markers characteristic of thymus-derived T lymphocytes, and, as the disease progresses, a single T cell clone consisting of these cells often becomes dominant. Further progression of the disease is marked by loss of epidermotrophism, development of tumors comprised of atypical T cells, and visceral involvement by these cells leading eventually to the patient’s demise. On the basis of these facts, these disorders have been classified as lymphomas of T lymphocytes.

Keywords

Mycosis Fungoides Hodgkin Disease Sezary Syndrome Cutaneous Lymphoma Inflammatory Dermatosis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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© Springer Science+Business Media New York 1994

Authors and Affiliations

  • W. Clark Lambert
    • 1
  1. 1.Department of Laboratory Medicine and Pathology Department of DermatologyUMDNJ-New Jersey Medical SchoolNewarkUSA

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