Blood classification and blood response criteria in mycosis fungoides and Sézary syndrome using flow cytometry: recommendations from the EORTC cutaneous lymphoma task force

Eur J Cancer. 2018 Apr;93:47-56. doi: 10.1016/j.ejca.2018.01.076. Epub 2018 Feb 21.


Our current mycosis fungoides (MF) and Sézary Syndrome (SS) staging system includes blood-classification from B0-B2 for patch/plaque/tumour or erythroderma based on manual Sézary counts but results from our EORTC survey confirm these are rarely performed in patch/plaque/tumour MF, and there is a trend towards using flow cytometry to measure blood-class. Accurately assigning blood-class effects overall stage and the 'global response' used to measure treatment responses in MF/SS and hence impacts management. The EORTC Cutaneous Lymphoma Task Force Committee have reviewed the literature and held a Workshop (June 2017) to agree a definition of blood-class according to flow cytometry. No large study comparing blood-class as defined by Sézary count with flow cytometry has been performed in MF/SS. The definition of blood-class by flow cytometry varies between publications. Low-level blood involvement occurs in patch/plaque/tumour much less than erythroderma (p < 0.001). The prognostic relevance of blood involvement (B1 or B2) in patch/plaque/tumour is not known. Studies have not shown a statistically worse difference in prognosis in erythrodermic MF patients with low-level blood involvement (IIIB) versus those without (IIIA), but Sezary patients who by definition have a leukaemic blood picture (staged IVA1 or higher) have a worse prognosis. For consistency flow, definition for blood-class must be an objective measurement. We propose absolute counts of either CD4+CD7-or CD4+CD26-where B0<250/μL, B1 = 250/μl-<1000/μL and B2≥1000/μL plus a T-cell blood clone. Fluctuations between B0 and B1 should not be considered in the treatment response criteria until further prognostic information is known.

Keywords: Blood; CD26; CD7; Classification; Cutaneous T-cell lymphoma; Erythroderma; Staging.

Publication types

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

MeSH terms

  • Flow Cytometry / methods*
  • Humans
  • Lymphoma / blood
  • Lymphoma / classification
  • Lymphoma / pathology*
  • Mycosis Fungoides / blood
  • Mycosis Fungoides / classification
  • Mycosis Fungoides / pathology*
  • Neoplasm Staging
  • Sezary Syndrome / blood
  • Sezary Syndrome / classification
  • Sezary Syndrome / pathology*
  • Skin Neoplasms / blood
  • Skin Neoplasms / classification
  • Skin Neoplasms / pathology*