Sézary syndrome

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 2B02

Sézary & Bouvrain 1938.

Aggressive, leukaemic cutaneous T-cell lymphoma.

  • First manifestation usually between 5th-7th decade of life.
  • Men > women.

  • Unknown.
  • May be the erythrodermic variant of mycosis fungoides.

  • Slightly scaly, pruritic erythema that progresses to erythroderma. The skin lesions tend to hyperpigment, which is why it is referred to as melanoerythroderma
  • Characteristic are light, skin-coloured macules, which are localised in the skin lesions. They are called nappes claires.
  • Edema (anasarca)
  • Facies-leontina
  • Palmoplantar hyperkeratosis is visible in almost half of all patients. Diffuse alopecia and onychodystrophy may occur
  • Locoregional lymphadenopathy
  • Bone marrow involvement (rare).

  • Clinic
  • Laboratory
    • BSG/CRP, diff.-BB (leukocytosis, rel. lymphocytosis), liver and kidney values, LDH, electrolytes.
    • Sézary cells (= Lutzner cells)
      • At > 1000 cells/μL à Sézary syndrome
    • FACS analysis, CD4/CD8 ratio, determination of CD4+CD7- cells
    • Clonality detection in blood
    • Bone marrow biopsy is usually not indicated
    • Immunelectrophoresis, if necessary
    • If necessary, HTLV serology
    • If necessary, borrelia serology
  • Biopsy
    • Dermatopathology
    • Immunohistology:
      • CD4+/CD7- T cells > 40% or CD4/CD8 ratio > 10
      • T helper phenotype: CD3+, CD4+, CD8-.
      • Molecular biology: clonal rearrangment of T-cell receptor genes.

For staging, see latest SOP.

  • Routine histology and immunopathology similar to MF.
  • In 40% of cases, histology is not diagnostic.
  • Monoclonal T cell population visible subepidermally.
  • Characteristic are atypical lymphocytes with highly lobulated nuclei (Lutzner cells).
  • Pauterised abscesses may be visible.
  1. Erratum in Olsen et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110:1713-1722. Blood, 2008. 111(9): p. 4830-4830.
  2. Beylot-Barry, M., et al., Is bone marrow biopsy necessary in patients with mycosis fungoides and Sezary syndrome? A histological and molecular study at diagnosis and during follow-up. Br J Dermatol, 2005. 152(6): p. 1378-1379.
  3. Booken, N., et al., Die Kombinationstherapie mit extrakorporaler Photopherese, Interferon-α, PUVA und lokalen Glukokortikoiden in der Behandlung des Sézary-Syndroms. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 2010. 8(6): p. 428-438.
  4. Bunn, P.A., Systemic Therapy of Cutaneous T-Cell Lymphomas (Mycosis Fungoides and the Sezary Syndrome). Annals of Internal Medicine, 1994. 121(8): p. 592.
  5. Caprini, E., et al., Identification of Key Regions and Genes Important in the Pathogenesis of Sezary Syndrome by Combining Genomic and Expression Microarrays. Cancer Research, 2009. 69(21): p. 8438-8446.
  6. Dummer, R., et al., Aktuelle Aspekte zur Pathogenese von Sézary-Syndrom und Mycosis fungoides. Der Hautarzt, 2001. 52(3): p. 189-192.
  7. Introcaso, C.E., et al., Total skin electron beam therapy may be associated with improvement of peripheral blood disease in Sézary syndrome. Journal of the American Academy of Dermatology, 2008. 58(4): p. 592-595.
  8. Kim, Y.H., et al., TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood, 2007. 110(2): p. 479-484.
  9. Prince, H.M., S. Whittaker, and R.T. Hoppe, How I treat mycosis fungoides and Sezary syndrome. Blood, 2009. 114(20): p. 4337-4353.
  10. Richardson, S.K., et al., High Clinical Response Rate with Multimodality Immunomodulatory Therapy for Sézary Syndrome. Clinical Lymphoma and Myeloma, 2006. 7(3): p. 226-232.
  11. Scarisbrick, J.J., et al., Secondary Malignant Neoplasms in 71 Patients With Sézary Syndrome. Arch Dermatol, 1999. 135(11).
  12. Schappell, D.L., Treatment of Advanced Mycosis Fungoides and Sézary Syndrome With Continuous Infusions of Methotrexate Followed by Fluorouracil and Leucovorin Rescue. Arch Dermatol, 1995. 131(3): p. 307.
  13. Talpur, R., R. Bassett, and M. Duvic, Prevalence and treatment of Staphylococcus aureus colonization in patients with mycosis fungoides and Sézary syndrome. Br J Dermatol, 2008. 159(1): p. 105-112.
  14. Trautinger, F., et al., EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. European Journal of Cancer, 2006. 42(8): p. 1014-1030.
  15. Trotter, M.J., et al., Cutaneous histopathology of Sézary syndrome: a study of 41 cases with a proven circulating T-cell clone. Journal of Cutaneous Pathology, 1997. 24(5): p. 286-291.
  16. van Doorn, R., et al., Oncogenomic analysis of mycosis fungoides reveals major differences with Sezary syndrome. Blood, 2008. 113(1): p. 127-136.
  17. Vermeer, M.H., et al., Novel and Highly Recurrent Chromosomal Alterations in Sezary Syndrome. Cancer Research, 2008. 68(8): p. 2689-2698.
  18. Whittaker, S.J. and F.M. Foss, Efficacy and tolerability of currently available therapies for the mycosis fungoides and Sezary syndrome variants of cutaneous T-cell lymphoma. Cancer Treatment Reviews, 2007. 33(2): p. 146-160.
  19. Wood, G.S., et al., Detection of Clonal T-Cell Receptor γ Gene Rearrangements in Early Mycosis Fungoides/Sezary Syndrome by Polymerase Chain Reaction and Denaturing Gradient Gel Electrophoresis (PCR/DGGE). Journal of Investigative Dermatology, 1994. 103(1): p. 34-41.
  20. Stadler, R., et al., Short German guidelines: cutaneous lymphomas. J Dtsch Dermatol Ges, 2008. 6 Suppl 1: p. S25-31.