Determination of immunophenotypic aberrancies provides better assessment of peripheral blood involvement by mycosis fungoides/Sézary syndrome than quantification of CD26− or CD7− CD4+ T-cells

Kirill A. Lyapichev, Ismael Bah, Auris Huen, Madeleine Duvic, Mark J. Routbort, Wei Wang, Jeffrey L. Jorgensen, L. Jeffrey Medeiros, Francisco Vega, Fiona E. Craig, Sa A. Wang

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

Background: Blood involvement by mycosis fungoides (MF)/Sézary syndrome (SS) influences prognosis and therapeutic decisions. MF/SS blood stage is currently determined by absolute CD4 + CD26− or CD4 + CD7-cell counts, which quantification method may overestimate MF/SS by including CD26− or CD7− normal CD4+ T-cells, or underestimate disease burden when MF/SS cells show incomplete loss of CD26 and/or CD7. Recently, through the standardization effort led by the International Clinical Cytometry Society (ICCS), recommendation was made to quantify MF/SS by enumerating immunophenotypically aberrant CD4+ T-cells, rather than CD26− or CD7− in isolation. Methods: We compared these two quantitation methods in 309 MF/SS patients who had blood samples analyzed by flow cytometry immunophenotyping (FCI) over a 1-year period. Results: Using the European Organization of Research and Treatment of Cancer (EORTC)/International Society for Cutaneous Lymphomas (ISCL) criteria, 221 (71.5%) patients had a blood stage corresponding to B0, 57 (18.4%) to B1, and 31 (10%) to B2. By FCI analysis, a total of 62 patients (20.0%) were found positive for MF/SS. Among EORTC B0 patients, 11/221 (5%) were positive by FCI (false negatives), and among EORTC Stage B1 patients, 35/57 (61%) were negative by FCI (false positives). Regarding patients positive for MF/SS cells by FCI, there was an overall excellent correlation (r =.999, p <.001) between the EORTC/ISCL method and FCI method; however, four (6.5%) patients would have an altered B stage between B0 and B1. Conclusion: The MF/SS cell quantification method using immunophenotypic aberrancies, as recommended by the ICCS, allows to distinguish MF/SS cells from background benign T-cells and enables for more accurate staging, especially among patients currently being considered to have B0 and B1 stage diseases.

Original languageEnglish (US)
Pages (from-to)183-191
Number of pages9
JournalCytometry Part B - Clinical Cytometry
Volume100
Issue number2
DOIs
StatePublished - Mar 2021
Externally publishedYes

Keywords

  • Sézary syndrome
  • blood staging
  • flow cytometry
  • mycosis fungoides

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Cell Biology

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