Oncology & Hematology Coding Alert

Define Mycosis Fu ngoides and Sézary disease

Check atypical cells in blood and lymphadenopathy in patient with a suspected red rash.

T-cell lymphomas with primary manifestations in the skin include Sézary disease and mycosis fungoides. The cell of origin in these lymphomas is the peripheral CD4+ T-lymphocyte. Due to the epidermal location of the neoplastic T cells, there is formation of small abscesses called Pautrier’s microabscesses which are the hallmark of the disease. Sézary disease often is described as a late stage of mycosis fungoides accompanied by lymphadenopathy.

Key signs and symptoms of these cutaneous T-cell lymphomas include:

  1. Generalized erythroderma or red rash of the skin.
  2. Lymphadenopathy.
  3. Atypical T-cells (“Sézary cells”) in the peripheral blood are seen in Sézary disease.
  4. Enlargement of the liver and spleen.

The red rash of mycosis fungoides: The skin may develop a scaly red rash which may be asymptomatic for many months. This may advance to an eczematous rash followed by a papular rash on hard skin. Finally, the tumors form in the skin with ulcers which may become infected.

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