Long-term Outcome of 525 Patients With Mycosis Fungoides and Sézary Syndrome

Kim, Youn H.; Liu, Howard L.; Mraz-Gernhard, Serena; Varghese, Anna; Hoppe, Richard T.

doi:10.1001/archderm.139.7.857

Cited by 571 publications

(466 citation statements)

References 36 publications

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“…1 However, one must bear in mind that many patients with SS are classified as clinical stage IV disease if they have extracutaneous involvement, a group known to have poor outcome. 3,16 Indeed, in our analysis, patients with extracutaneous (stage IV) disease had the poorest outcomes.…”

Section: Discussionmentioning

confidence: 73%

“…Because the MFCG staging system does not take blood involvement into account when determining overall clinical stage, patients with SS are generally staged as stage III, unless lymph node (stage IVA) or visceral disease (stage IVB) is present. Previous studies have demonstrated that the prognosis of advanced-stage disease (ASD) is generally poor 3,4 ; median overall survival (OS) in patients with tumor, erythrodermic, and extracutaneous disease have been reported to be 40, 48, and 13 months, respectively.…”

Section: Introductionmentioning

confidence: 99%

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Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation

Arulogun

Prince

et al. 2008

Blood

165

141

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Section: Discussionmentioning

confidence: 73%

Section: Introductionmentioning

confidence: 99%

Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation

Arulogun

Prince

et al. 2008

Blood

165

141

View full text Add to dashboard Cite

“…Many patients will have had symptoms attributed to eczema or parapsoriasis for years before obtaining a definitive diagnosis. The median time from symptom onset to diagnosis in retrospective series is 3-4 years, but may exceed four decades [99][100][101]. Clinically, patch/plaque stage MF is frequently characterized by persistent and progressive lesions that develop in a ''bathing suit'' distribution and vary in size, shape and color.…”

Section: Mycosis Fungoidesmentioning

confidence: 99%

“…Patients with patch/plaque stage disease (T1/T2) and architectural preservation of any clinically abnormal lymph nodes are classified as stage IIA. Collectively, patients with stage I-IIA disease have ''limited-stage'' disease, as the overall survival in these patients is measured in decades, with survival in patients with stage IA disease resembling that of normal age-matched controls [7,99,100]. At diagnosis, the majority of MF patients will have limitedstage disease [7].…”

Section: Risk-stratification Stagingmentioning

confidence: 99%

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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“…2 However, in recent years, lymphoma cells in the peripheral blood, especially at high levels, has been recognized as an independent adverse prognostic indicator in patients with mycosis fungoides and Sézary syndrome. [3][4][5] Therefore, the International Society for Cutaneous Lymphomas and the Cutaneous Lymphoma Task Force of European Organization for Research and Treatment of Cancer revised the mycosis fungoides/Sézary syndrome staging criteria in 2007 to incorporate blood (B) involvement as a major prognostic factor, and defined the criteria for B-staging. 6 When assessing B-stage, one critical issue is the optimal method used to identify and quantify lymphoma cells.…”

mentioning

confidence: 99%

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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Long-term Outcome of 525 Patients With Mycosis Fungoides and Sézary Syndrome

Abstract: To study and update the clinical characteristics and long-term outcome of our patients with mycosis fungoides (MF) and Sézary syndrome (SS), and to identify important clinical factors predictive of survival and disease progression.

Cited by 571 publications

References 36 publications

Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation

Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation

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

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