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Comparative Study
. 2016 May;74(5):870-7.
doi: 10.1016/j.jaad.2015.12.018. Epub 2016 Feb 10.

Cutaneous T-cell lymphoma (CTCL): Current practices in blood assessment and the utility of T-cell receptor (TCR)-Vβ chain restriction

Juliet F Gibson  1 , Jing Huang  2 , Kristina J Liu  3 , Kacie R Carlson  1 , Francine Foss  4 , Jaehyuk Choi  1 , Richard Edelson  1 , Jerry W Hussong  5 , Ramsey Mohl  5 , Sally Hill  5 , Michael Girardi  6
Affiliations
Comparative Study

Cutaneous T-cell lymphoma (CTCL): Current practices in blood assessment and the utility of T-cell receptor (TCR)-Vβ chain restriction

Juliet F Gibson et al. J Am Acad Dermatol. 2016 May.

Abstract

Background: Accurate quantification of malignant cells in the peripheral blood of patients with cutaneous T-cell lymphoma is important for early detection, prognosis, and monitoring disease burden.

Objective: We sought to determine the spectrum of current clinical practices; critically evaluate elements of current International Society for Cutaneous Lymphomas (ISCL) B1 and B2 staging criteria; and assess the potential role of T-cell receptor-Vβ analysis by flow cytometry.

Methods: We assessed current clinical practices by survey, and performed a retrospective analysis of 161 patients evaluated at Yale (2011-2014) to compare the sensitivity, specificity, positive predictive value, and negative predictive value of parameters for ISCL B2 staging.

Results: There was heterogeneity in clinical practices among institutions. ISCL B1 criteria did not capture 5 Yale cohort cases with immunophenotypic abnormalities that later progressed. T-cell receptor-Vβ testing was more specific than polymerase chain reaction and aided diagnosis in detecting clonality, but was of limited benefit in quantification of tumor burden.

Limitations: Because of limited follow-up involving a single center, further investigation will be necessary to conclude whether our proposed diagnostic algorithm is of general clinical benefit.

Conclusion: We propose further study of modified B1 criteria: CD4/CD8 ratio 5 or greater, %CD4(+) CD26(-) 20% or greater, or %CD4(+) CD7(-) 20% or greater, with evidence of clonality. T-cell receptor-Vβ testing should be considered in future diagnostic and staging algorithms.

Keywords: Sézary syndrome; T-cell receptor-Vβ; cutaneous T-cell lymphoma; flow cytometry; mycosis fungoides; peripheral blood analysis.

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Conflict of interest statement

Conflicts of interest: Dr. Hussong is the Director of Laboratory for ARUP Laboratories, Salt Lake City, UT; is on the Executive Committee for ARUP Laboratories, Salt Lake City, UT. Mr. Mohl is a technical supervisor for ARUP Laboratories, Salt Lake City, UT. Ms. Hill is a hematologic flow cytometry specialist for ARUP Laboratories, Salt Lake City, UT.

Figures

Figure 1
Figure 1. Proposed algorithm for assessment of blood involvement in CTCL
This algorithm may aid in further research investigating the prognostic significance of our proposed modified B1 criteria and “equivocal” TCR-Vβ tests. As loss of CD26 was the most sensitive parameter of the current B2 criteria, we would suggest investigating the potential for TCR-Vβ testing in CD4+CD26- populations. B1 and B2 were defined by ISLC criteria (if atypical lymphocytes were noted based on immunophenotypic alterations only, this percentage was multiplied by the absolute lymphocyte count to approximate the number of “Sézary cells” where no such counts were performed/available).
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