Mycosis fungoides pathophysiology

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Cutaneous T cell lymphoma Microchapters

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Overview

Classification

Mycosis fungoides
Sezary syndrome

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Cutaneous T cell lymphoma arises from T-cells. On microscopic histopathological analysis, atypical lymphoid cells, polymorphous inflammatory infiltrate in the dermis, and lymphocytes with cerebroid nuclei are characteristic findings of mycosis fungoides.

Pathophysiology

  • Cutaneous T cell lymphoma is an unusual expression of T-cells, a part of the immune system
  • These T-cells are skin-associated, meaning that they biochemically and biologically are most related to the skin, in a dynamic manner
  • Sezary syndrome and Mycosis Fungoides are T-cell lymphomas whose primary manifestation is in the skin
  • Mycosis Fungoides is the most common type of 'Cutaneous T cell lymphoma' (CTCL)
  • Mycosis fungoides is initially an indolent lymphoma but in its later stages can cause peripheral lymphadenopathy and can finally progress to widespread extracutaneous visceral / internal organ involvement
  • "Sézary's cells" are T-cells that have pathological quantities of mucopolysaccharides
  • Sézary's disease is sometimes considered a late stage of mycosis fungoides

Gross Pathology

Microscopic Pathology

  • Mycosis fungoides has been divided into three stages:
  • Premycotic stage
  • Mycotic stage
  • Tumorous stage
  • The premycotic stage
  • Non-diagnostic and represented by chronic nonspecific dermatisis associated with psoriasiform changes in epidermis
  • The mycotic stage
  • Shows a polymorphous inflammatory infiltrate in the dermis that contains small numbers of frankly atypical lymphoid cells
  • These cells may line up individually along the epidermal basal layer
  • The latter finding if unaccompanied by spongiosis is highly suggestive of mycosis fungoides
  • Tumorous stage
  • Dense infiltrate of medium sized lymphocytes with cerebroid nuclei, expands the dermis


References


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