Evaluation of lymphangiogenic markers in Sézary syndrome

Leuk Lymphoma. 2011 Mar;52(3):491-501. doi: 10.3109/10428194.2010.517877. Epub 2010 Sep 17.


Sézary syndrome (SS) is regarded as a leukemic, aggressive subtype of cutaneous T-cell lymphoma (CTCL) characterized by the accumulation of malignant T-cells in the skin, as well as by blood and lymph node involvement. To date there have been no data on the extent of lymphangiogenesis in SS or erythrodermic mycosis fungoides (eMF). Lymphangiogenesis represents the de novo formation of lymphatic vasculature and has been associated with the occurrence of metastatic disease and poor prognosis. In this study we investigated lymphangiogenesis in skin biopsies from patients with SS and eMF. The expression of VEGFR-3 was significantly higher in patients with SS (p = 0.0285) as compared to patients with eMF. LYVE-1, podoplanin (PDPN), and VEGF-C stainings showed a similar tendency. The number of PDPN-expressing lymphatic vessels (p = 0.025) as well as CD31-positive blood vessels (p = 0.0065) correlated with disease progression in patients with SS. We show for the first time a non-vascular pattern of VEGF-C and VEGFR-3, i.e. their epidermal expression in erythrodermic CTCLs, suggesting their role in lymphocyte trafficking to the skin.

Publication types

  • Evaluation Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Biomarkers, Tumor / analysis*
  • Biomarkers, Tumor / metabolism
  • Biopsy
  • Cohort Studies
  • Disease Progression
  • Female
  • Humans
  • Immunohistochemistry
  • Lymph Nodes / metabolism
  • Lymph Nodes / pathology
  • Lymphangiogenesis* / physiology
  • Lymphatic Vessels / metabolism*
  • Lymphatic Vessels / pathology
  • Male
  • Middle Aged
  • Mycosis Fungoides / diagnosis
  • Mycosis Fungoides / metabolism
  • Mycosis Fungoides / pathology
  • Prognosis
  • Sezary Syndrome / diagnosis
  • Sezary Syndrome / metabolism*
  • Sezary Syndrome / pathology
  • Skin Neoplasms / diagnosis
  • Skin Neoplasms / metabolism*
  • Skin Neoplasms / pathology


  • Biomarkers, Tumor