Cutaneous T-Cell Lymphoma

Aka: Cutaneous T-Cell Lymphoma, Mycosis Fungoides, Sezary Syndrome, Sezary Cell, Sezary Erythroderma, Hypopigmented Mycosis Fungoides

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II. Pathophysiology

  1. T-Cell Lymphoma that begins in skin
  2. Sezary Syndrome is leukemic form of T-Cell Lymphoma
  3. Hypopigmented Mycosis Fungoides is a variant found in children
    1. Hypopigmented nonatrophic patches with fine scale, found on the trunk, buttocks and extremities
    2. CD8 positive (negative in typical Mycosis Fungoides)
    3. Unlike typical Mycosis Fungoides, skin findings may be only manifestation and has a favorable prognosis
    4. Treatment with Phototherapy, Immunotherapy, UV radiation, Chemotherapy and topicals (e.g. steroids, Retinoids)
    5. Jung (2021) JAMA Dermatol 157(4): 431-8 [PubMed]
    6. Castano (2013) J Cutan Pathol 40(11): 924-34 [PubMed]

III. Epidemiology

  1. Incidence: 0.42 cases per 100,000 (rare)
  2. Most common after age 50 years
  3. Gender: Twice as common in men
  4. Black patients are more commonly affected

IV. Symptoms

  1. Pruritus

V. Signs (four stages of development)

  1. Eczematous patches and Plaques
  2. Distributed over non-sun-exposed skin
    1. Buttocks
    2. Thighs
  3. Tumors develop later in course
    1. Associated with superficial and deep Lymphadenopathy
    2. Associated with metastases to Spleen, lung, GI Tract

VI. Labs

  1. Histology
    1. Epidemotropism or Lymphocytes
    2. Perivascular and periadnexal infiltrate
    3. Halo around Lymphocytes
    4. Patchy lichenoid infiltrate
    5. Psoriasiform epidermal hyperplasia
    6. Dermal melanophages
  2. Other markers
    1. Expresses CD2, CD3, CD4 and CD5
      1. Typical Mycosis Fungoides does not express CD8
      2. Hypopigmented variant does express CD8
    2. PCR Testing
      1. Identifies T Cell gene rearrangements

VII. Complications

  1. Exfoliative Dermatitis

VIII. Course

  1. Survival <3 years after tumors develop

IX. References

  1. Habif (1996) Clinical Dermatology, Mosby, p. 674-5
  2. Pujol (2000) J Am Acad Dermatol 42:324-8 [PubMed]

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