Blood Flow Cytometry in Sézary Syndrome

Novelli, Mauro; Fava, Paolo; Sarda, Cristina; Ponti, Renata; Osella‐Abate, Simona; Savoia, Paola; Bergallo, Massimiliano; Lisa, Francesco; Fierro, Maria Teresa; Quaglino, Pietro

doi:10.1309/ajcp1na3ychcdeig

Cited by 47 publications

(21 citation statements)

References 44 publications

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“…The prognostic value of a cell surface marker may vary with treatment. Novelli et al., found SzS patients who had a presence of a variable proportion of CD26+ atypical cells at diagnosis showed a statistically significant higher overall survival. However, in their retrospective cohort study of eleven MF/SzS patients who had undergone treatment, Vandersee et al .…”

Section: Cell Surface Markersmentioning

confidence: 98%

“…Novelli et al, [50] found SzS patients who had a presence of a variable proportion of CD26+ atypical cells at diagnosis showed a statistically significant higher overall survival. However, in their retrospective cohort study of eleven MF/SzS patients who had undergone treatment, Vandersee et al [51] calculated a low positive predictive value for changes in CD26 expression and clinically meaningful events.…”

Section: Cell Surface Markersmentioning

confidence: 99%

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The biomarker landscape in mycosis fungoides and Sézary syndrome

Dulmage

Geskin

Guitart

et al. 2017

Experimental Dermatology

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The practice of pre-emptive individualized medicine is predicated on the discovery, development and application of biomarkers in specific clinical settings. Mycosis fungoides and Sézary syndrome are the two most common type of cutaneous T-cell lymphoma, yet diagnosis, prognosis and disease monitoring remain a challenge. In this review, we discuss the current state of biomarker discovery in mycosis fungoides and Sézary syndrome, highlighting the most promising molecules in different compartments. Further, we emphasize the need for continued multicentre efforts to validate available and new biomarkers and to develop prospective combinatorial panels of already discovered molecules.

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Section: Cell Surface Markersmentioning

confidence: 98%

Section: Cell Surface Markersmentioning

confidence: 99%

The biomarker landscape in mycosis fungoides and Sézary syndrome

Dulmage

Geskin

Guitart

et al. 2017

Experimental Dermatology

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“…With the recognition that neoplastic T cells have diminished expression of CD26 in about 90% of Sézary patients, the percentage and absolute numbers of CD4 + CD26lymphocytes have recently been proposed to define B ratings [18,21]. However, because CD26 may be variably expressed (some neoplastic cells positive, some negative), it is useful to also measure CD4 + CD7cells and to use this value if it exceeds the percentage of CD4 + CD26cells [27].…”

Section: Discussionmentioning

confidence: 99%

Prognostic Implications of Blood B Ratings for Erythrodermic Cutaneous T cell Lymphoma

Vonderheid¹

2018

J Clin Exp Dermatol Res

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Objective: The magnitude of blood involvement in cutaneous T cell lymphoma (CTCL) has prognostic significance. Several groups have proposed hematologic criteria for B ratings for use in clinical staging of CTCL, but in practice B ratings are only relevant for the staging of erythrodermic CTCL without lymph node or visceral involvement, i.e., T4N0-2M0B0 defines sub-stage IIIA, T4N0-2M0B1 defines sub-stage IIIB and T4N0-2M0B2 defines sub-stage IVA1. This retrospective study examines the effect of various B rating criteria on prognoses of patients with erythrodermic CTCL. Methods: At initial presentation, 152 patients diagnosed with E-CTCL had quantitative Sézary cell counts performed on blood smears and CD4/CD8 ratios determined in the blood by flow cytometry. In addition, 39 patients had percentages of CD4 + CD26and CD4 + CD7lymphocytes directly measured. The Kaplan-Meier method and Cox proportional hazards model were used to estimate mean overall and CTCL-specific survival and to compare survival curves of sub-stages of erythrodermic CTCL. Results: With B2 rating defined as absolute Sézary cell counts ≥ 1.0 K/µL or CD4/CD8 ratio ≥ 10, a better separation of survival curves between sub-stages IIIA and IIIB was observed if the threshold for B1 was defined as Sézary cells ≥ 20% and CD4/CD8 ratio defined as ≥ 4 plus confirmation of blood involvement. With either criterion, the survival of patients at sub-stages IVA1 and IVA2 were not statistically different. There was also a suggestion that percentage of CD4 + CD26or CD4 + CD7cells (which ever was higher) might be used to define B1 and B2 thresholds (≥ 30% and ≥ 60%, respectively) as an alternative to absolute counts. Maximum CD4 + CD26-/CD4 + CD7-≥ 30% plus confirmation also might provide a hematologic definition of leukemic CTCL. Conclusion: Additional studies on larger number of patients and evaluation with other measures of blood tumor burden are indicated.

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“…In addition to demonstrating clonally related neoplastic T cells in the skin and PB, one or more of the following criteria are required to diagnose SS: an absolute Sézary cell count ≥1,000/µL; an expanded CD4 + T-cell population resulting in a CD4 : CD8 ratio of ≥10; or the loss of one or more T-cell antigens. Because of the morphologic heterogeneity of Sézary cells, flow cytometric analysis of PB is essential for diagnostic purposes and tumor burden monitoring during follow-up [2,3]. Typical Sézary cells have a CD3 + CD4 + CD8phenotype and characteristically lack CD7 and CD26 [4].…”

Section: Introductionmentioning

confidence: 99%

Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood

Kageyama

Tsuda

Nato

et al. 2021

Case Reports in Hematology

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Sézary syndrome is a rare leukemic type of cutaneous T-cell lymphoma characterized by the presence of neoplastic T cells with cerebriform nuclei (Sézary cells) in the skin, lymph nodes, and peripheral blood. Typical Sézary cells have a CD3+CD4+CD8– phenotype; however, in cases of the aberrant loss of antigens on Sézary cells, especially the loss of critically important T-cell antigens such as CD4, there is a possibility of misdiagnosing the disease or underestimating the tumor burden of the disease. Here, we report a rare case of Sézary syndrome with CD4/CD8 double-negative Sézary cells in the peripheral blood. Most of the Sézary cells in the peripheral blood had lost CD4 expression, and we diagnosed the disease and evaluated the tumor burden by multicolor flow cytometry. Intriguingly, the Sézary cells showed a typical CD4+CD8–CD7– phenotype in the skin even though the cells in the peripheral blood lacked CD4. The patient responded well to treatment with bexarotene and narrow-band ultraviolet B therapy. Analysis by multicolor flow cytometry is essential to diagnose this rare type of Sézary syndrome and evaluate the tumor burden.

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Blood Flow Cytometry in Sézary Syndrome

Abstract: We confirm the relevance of CD26 negativity in SS diagnosis and monitoring. Nevertheless, the presence of rare CD26+ cases suggests that a multiparameter flow cytometry approach should be used. Changes in methylation profile could account for phenotypical heterogeneity.

Cited by 47 publications

References 44 publications

The biomarker landscape in mycosis fungoides and Sézary syndrome

The biomarker landscape in mycosis fungoides and Sézary syndrome

Prognostic Implications of Blood B Ratings for Erythrodermic Cutaneous T cell Lymphoma

Sézary Syndrome with CD4/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood

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