Fast Five Quiz: Sézary Syndrome

Lauren C. Pinter-Brown, MD


November 01, 2021

In contrast to many other lymphoproliferative disorders, in which cytogenetic and laboratory findings play a prominent role in risk stratification, TNMB (tumor, node, metastasis, blood) staging remains an important prognostic factor in Sézary syndrome (and mycosis fungoides), and forms the basis for a "risk-adapted" approach to treatment.

The staging is based on the particular skin findings and findings of extracutaneous disease:

  • Stage IA disease (as defined by the TNMB system): Patchy or plaque-like skin disease involving < 10% of skin surface area (T1 skin disease)

  • Stage IB: Patchy or plaque-like skin disease involving ≥ 10% of the skin surface area (T2 skin disease)

  • Stage IIB disease: Tumors are present (T3 skin lesions)

  • Stage III disease: Generalized erythroderma is present

  • Stage IVA1 disease: Erythroderma and significant blood involvement occur 

  • Stage IVA2 disease: Lymph node biopsy shows total effacement by atypical cells (LN4 node) 

  • Stage IVB disease: Visceral involvement (eg, liver, lung, bone marrow) occurs

Learn more about staging of Sézary syndrome.

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