Sézary Syndrome

Sézary Syndrome

 

Sézary syndrome represents the disseminated leukemic form of the primary cutaneous T-cell lymphoma (CTCL) known as mycosis fungoides (MF).

Epidemiology

  • Incidence – <0.5/100,000
  • Age – median age of onset in 6th decade
  • Gender – M>F
  • Ethnicity – occurs more frequently in blacks

Pathophysiology

  • Considered to be caused by malignant T-helper cells in dynamic equilibrium between the skin and vascular compartments

Clinical Presentation

  • Erythroderma, edema, pruritus, adenopathy, ectropion, keratoderma
  • Most commonly, signs and symptoms arise de novo but can follow nonspecific dermatitis or, rarely, MF
  • Peripheral blood involvement of circulating malignant lymphoma cells (Sézary cells)
  • Patients diagnosed with Sézary syndrome generally have a more advanced disease stage and worse prognosis than those diagnosed with classic MF localized to skin

Diagnosis

  • Laboratory testing
    • Sézary cell exam
      • Sézary cells are abnormal lymphocytes which undergo nuclear, but not cytoplasmic, division
      • Sézary cells can be seen in small numbers in normal peripheral blood
      • Sézary cells >12 μm in diameter and ≥20%/100 lymphocytes is diagnostic
    • Flow cytometric analysis
      • Detect and quantify phenotypically abnormal T-cell population (usually CD4+, CD7-, CD26 – see Leukemia Lymphoma phenotyping algorithm)
    • T-cell clonality studies
      • Confirm diagnosis of Sézary cell

See Also