The diagnosis of Sézary syndrome on peripheral blood by flow cytometry requires the use of multiple markers

Klemke, Claus‐Detlev; Brade, Joachim; Weckesser, Steffi; Sachse, Michael Max; Booken, Nina; Neumaier, Michael; Goerdt, Sergij; Nebe, Thomas

doi:10.1111/j.1365-2133.2008.08739.x

Cited by 65 publications

(75 citation statements)

References 46 publications

(95 reference statements)

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“…Molecular studies, including detection of a clonal TCR gene rearrangement by PCR and the presence of a clonal cytogenetic abnormality, provide evidence of T-cell clonality. An alternative approach to demonstrate T-cell clonality incorporates multicolor flow cytometry using a panel of antibodies specific for various TCR beta-chain variable region family members (TCR-Vb) [140][141][142]. This approach is successful in identifying a clonal population of T cells if this population is significantly higher than the background frequency of polyclonal T cells harboring the same Vb chain [140,141].…”

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confidence: 99%

“…An alternative approach to demonstrate T-cell clonality incorporates multicolor flow cytometry using a panel of antibodies specific for various TCR beta-chain variable region family members (TCR-Vb) [140][141][142]. This approach is successful in identifying a clonal population of T cells if this population is significantly higher than the background frequency of polyclonal T cells harboring the same Vb chain [140,141]. Clark et al recently observed that lymphocytes isolated from either peripheral blood or skin lesions of CTCL patients contained a population of cells with high forward and side scatter characteristics on flow cytometric analysis [24].…”

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confidence: 99%

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Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

Wilcox

2011

American J Hematol

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Disease overview: Cutaneous T‐cell lymphomas are a heterogenous group of T‐cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis fungoides (MF) or Sézary syndrome (SS).Diagnosis: The diagnosis of MF or SS requires the integration of clinical and histopathologic data.Risk‐adapted therapy: Tumor, node, metastasis, and blood (TNMB) staging remains the most important prognostic factor in MF/SS and forms the basis for a “risk‐adapted,” multidisciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin‐directed therapies is preferred, as both disease‐specific and overall survival for these patients is favorable. In contrast, patients with advanced‐stage disease with significant nodal, visceral, or blood involvement are generally approached with biologic‐response modifiers, denileukin diftitox, and histone deacetylase inhibitors before escalating therapy to include systemic, single‐agent chemotherapy. Multiagent chemotherapy may be used for those patients with extensive visceral involvement requiring rapid disease control. In highly‐selected patients with disease refractory to standard treatments, allogeneic stem‐cell transplantation may be considered. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc.

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Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

Wilcox

2011

American J Hematol

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“…7,10 In the past decade, flow cytometric immunophenotyping has proven to be more reliable than morphologic examination in assessing for lymphoma (also known as Sézary) cells in peripheral blood of mycosis fungoides and Sézary syndrome patients, as the neoplastic cells often have an aberrant immunophenotype. [11][12][13][14][15][16] However, it has been recognized that T-cell antigenic alterations are not entirely specific for mycosis fungoides or Sézary syndrome, and have been observed in some reactive conditions. 11,17,18 Assessment of T-cell clonality using molecular methods is therefore recommended by the International Society for Cutaneous Lymphomas, even in the presence of flow cytometric immunophenotypic aberrancies.…”

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confidence: 99%

“…Subsequently, several studies validated and expanded the use of flow cytometry to assess T-cell receptor Vb in T-cell malignancies. 13,25,[27][28][29][30] Morice et al 30 studied 29 T-cell lymphoproliferative disorders by flow cytometric Vb analysis, including 10 cases of cutaneous T-cell lymphoma, and showed a good correlation with molecular methods. This group expanded their study to include 11 Sézary syndrome and 6 mycosis fungoides cases, and showed that Vb analysis by flow cytometry was helpful for assessing clonality and quantifying the peripheral blood tumor burden.…”

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Flow cytometric detection of peripheral blood involvement by mycosis fungoides and Sézary syndrome using T-cell receptor Vβ chain antibodies and its application in blood staging

et al. 2010

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Peripheral blood involvement has been recognized as an adverse prognostic factor in patients with mycosis fungoides and Sé zary syndrome. However, accurate identification and enumeration of the neoplastic cells in these diseases can be challenging. We assessed the clinical utility of flow cytometric immunophenotypic analysis of T-cell receptor Vb expression in 82 mycosis fungoides and 6 Sé zary syndrome patients, with an atypical T-cell immunophenotype, or abnormal CD4:CD8 ratio, identified from peripheral blood specimens of 723 patients submitted for routine mycosis fungoides/Sé zary syndrome blood staging. To improve detection sensitivity, Vb expression was analyzed on gated CD3 þ CD4 þ T cells or T cells with an aberrant immunophenotype, if present. The flow cytometric results were compared with traditional morphologic assessment (n ¼ 88) and molecular methods to assess the T-cell receptor c or b genes (n ¼ 41 tested in parallel). Flow cytometric immunophenotyping yielded a clonal Vb pattern in 60/82 mycosis fungoides and 6/6 Sé zary syndrome patients. By contrast, flow cytometric Vb was negative in all 10 healthy donors and 18 control patients, showing a specificity of 100% and concordance with molecular testing of 86%. Using flow cytometric Vb results instead of morphologic assessment, 12 patients were upstaged from B1 to B2, and 20 patients from B0 to B1 (Po0.0001). The 12 upstaged B2 patients had no morphologic evidence of involvement, but had an aggressive clinical course similar to those staged by traditional morphologic assessment (median survival 27 vs 41 months, log-rank P ¼ 0.701). In 30/44 patients with a tumor-associated Vb expression, a single Vb tube was used to monitor treatment response. In conclusion, flow cytometric Vb analysis is rapid and convenient, can assess T-cell clonality and tumor quantity simultaneously, and is useful both in initial blood staging and monitoring tumor burden during therapy in patients with mycosis fungoides or Sé zary syndrome.

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“…Molecular studies, including the detection of a clonal TCR gene rearrangement by PCR and the presence of a clonal cytogenetic abnormality, provide evidence of T-cell clonality. An alternative approach to demonstrate this clonality incorporates multicolor flow cytometry using a panel of antibodies that are specific for various TCR beta-chain variable region family members (TCR-Vβ) [10] [11]. The currently criteria recommended for the diagnosis of SS by the International Society of Cutaneous Lymphomas include the following: absolute Sezary count ≥1000/ml, a CD4/CD8 ratio ≥10, aberrant expression of pan-T-cell antigens, demonstration of T-cell clonality by Southern blot or PCR-based methods, or cytogenetic demonstration of an abnormal clone [5].…”

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confidence: 99%

MicroRNA-mediated Gene Expression in Sezary Syndrome: An Overview

Cristofoletti¹,

Picchio²,

Russo³

2015

ITI

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Abstract-Sezary syndrome (SS) is a rare leukemic cutaneous T-cell lymphoma (CTCL) with an aggressive clinical course. It is characterized by erythroderma, generalized lymphadenopathy and neoplastic skin-homing CD4+ memory T cells (Sezary cells) in the skin, lymph nodes, and peripheral blood. Despite the availability of a number of active systemic therapeutic strategies, SS remains an incurable lymphoma. Recently, high throughput analyses have revealed a novel approach to identify the molecular process implicated in the development and tumorigenesis of SS, which is relevant to a pharmacological therapeutic strategy. The aim of this review is to describe some altered genes, starting from the main deregulated microRNAs discovered in SS.

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The diagnosis of Sézary syndrome on peripheral blood by flow cytometry requires the use of multiple markers

Cited by 65 publications

References 46 publications

Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

Flow cytometric detection of peripheral blood involvement by mycosis fungoides and Sézary syndrome using T-cell receptor Vβ chain antibodies and its application in blood staging

MicroRNA-mediated Gene Expression in Sezary Syndrome: An Overview

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