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Updated: Dec 26 2021

Cutaneous T-Cell Lymphoma

  • Snapshot
    • A 60-year-old patient is at his dermatologist’s office for follow-up of his eczematous dermatitis. He has tried topical steroids with no improvement. He complains of increasing itchiness. On physical exam, he has multiple confluent and well-demarcated pink patches on his buttocks and legs with some scaling. His dermatologist suspects that this may not be eczematous dermatitis but something that mimics like it. He also has prominent inguinal lymph nodes. Prior skin biopsies have been undiagnostic, but his dermatologist decides to do one more skin biopsy. This time, the biopsy reveals classic Sézary cells.
  • Introduction
    • CD4+ helper T-cell lymphoma of the skin
    • Two variants of cutaneous T-cell lymphoma (CTCL)
      • mycosis fungoides (50-70% of CTCLs)
        • skin involvement only
      • Sézary syndrome (1-3% of CTCLs)
        • leukemic form of CTCL
        • CTCL plus malignant T-cells in the blood
    • Other less common variant
      • adult T-cell leukemia/lymphoma (ATLL)
  • Epidemiology
    • Older patients (median age 55-60)
    • More common in male patients
    • More common in black patients
  • etiology
    • Associated conditions
      • secondary malignancies
        • Hodgkin lymphoma
        • other non-Hodgkin lymphomas
        • melanoma
  • Presentation
    • Symptoms
      • generalized pruritus
    • Physical exam
      • mycosis fungoides
        • well-demarcated, erythematous pruritic patches and plaques
        • in non-sun-exposed areas
        • can have telangiectasias and ulceration
        • erythroderma (> 90% body involvement)
        • early lesions may be confused with atopic dermatitis of psoriasis
      • Sézary syndrome
        • severely pruritic erythroderma with scaling
        • alopecia
        • lymphadenopathy
        • hepatosplenomegaly
    • Imaging if leukemia suspected
      • CT or PET-CT of whole body
  • studies
    • Physical exam to assess for lymphadenopathy and organomegaly
    • Labs
      • complete blood count with peripheral blood smear to look for Sézary cells (atypical T-cells)
      • PCR of blood if leukemia suspected for atypical T-cells
  • Differential Diagnosis
    • Adult T-cell leukemia-lymphoma (ATLL)
    • Atopic dermatitis
    • Psoriasis
    • Drug eruption (SJS)
  • diagnosis
    • Diagnosis usually made by clinical exam and history
    • Diagnosis confirmed with skin biopsy
      • biopsy may be inconclusive with one lesion
      • several months with multiple biopsies may be needed to confirm diagnosis
        • lymphocytic infiltrate with microabscesses within thickened epidermis.
        • buttock cells”: atypical lymphocytes with cerebriform nuclei (Sézary cells)
  • Treatment
    • Stage-dependent
      • localized
        • topical steroids
        • topical chemotherapies
        • topical retinoids
        • PUVA
      • systemic
        • radiation therapy
        • systemic retinoids
        • interferon
        • methotrexate
  • Complications
    • Intense pruritus
    • Secondary cutaneous bacterial or viral infection
  • Prognosis
    • Stage-dependent
    • Mycosis fungoides typically indolent
      • slow-growing
    • Sézary syndrome typically aggressive
      • can be fatal
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