Prognostic factor analysis in mycosis fungoides/Sézary syndrome

Diamandidou, Eleni; Colomé, Maria I.; Fayad, Luis; Duvic, Madeleine; Kurzrock, Razelle

doi:10.1016/s0190-9622(99)70079-4

Cited by 163 publications

(140 citation statements)

References 25 publications

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“…In addition to staging, male gender, increasing age, an elevated LDH and the folliculotropic variant of MF were also independently associated with poorer overall and disease-specific survival. In contrast to previous reports highlighting the aggressive clinical course associated with large cell transformation [189][190][191][192][193], defined as the presence of large, atypical lymphocytes comprising at least 25% of the total lymphoid infiltrate, large cell transformation was not an independent predictor of overall or disease-specific survival but was associated with a higher risk (hazard ratio 3.32) of disease progression [7]. Given the importance of the TNMB classification in risk stratification and defining disease burden, the ISCL/EORTC recommends its use in defining the initial, maximum and current burden of disease, which will ultimately play an important role in the selection of either skin-directed or systemic therapies [143].…”

Section: Risk-stratification Stagingcontrasting

confidence: 85%

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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Section: Risk-stratification Stagingcontrasting

confidence: 85%

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

Wilcox

2011

American J Hematol

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“…These include clinical features such as male sex and older age, elevated lactate dehydrogenase (LDH), and histologic features of folliculotropism (FT) and large-cell transformation (LCT). [5][6][7][8][9][10][11] Previous studies of prognostic factors have been mainly single-center studies, and only a large-scale international collaboration will allow the true impact of these factors to be defined.…”

Section: Introductionmentioning

confidence: 99%

Cutaneous Lymphoma International Consortium Study of Outcome in Advanced Stages of Mycosis Fungoides and Sézary Syndrome: Effect of Specific Prognostic Markers on Survival and Development of a Prognostic Model

Scarisbrick

Prince

Vermeer

et al. 2015

JCO

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362

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Purpose Advanced-stage mycosis fungoides (MF; stage IIB to IV) and Sézary syndrome (SS) are aggressive lymphomas with a median survival of 1 to 5 years. Clinical management is stage based; however, there is wide range of outcome within stages. Published prognostic studies in MF/SS have been single-center trials. Because of the rarity of MF/SS, only a large collaboration would power a study to identify independent prognostic markers. Patients and Methods Literature review identified the following 10 candidate markers: stage, age, sex, cutaneous histologic features of folliculotropism, CD30 positivity, proliferation index, large-cell transformation, WBC/lymphocyte count, serum lactate dehydrogenase, and identical T-cell clone in blood and skin. Data were collected at specialist centers on patients diagnosed with advanced-stage MF/SS from 2007. Each parameter recorded at diagnosis was tested against overall survival (OS). Results Staging data on 1,275 patients with advanced MF/SS from 29 international sites were included for survival analysis. The median OS was 63 months, with 2- and 5-year survival rates of 77% and 52%, respectively. The median OS for patients with stage IIB disease was 68 months, but patients diagnosed with stage III disease had slightly improved survival compared with patients with stage IIB, although patients diagnosed with stage IV disease had significantly worse survival (48 months for stage IVA and 33 months for stage IVB). Of the 10 variables tested, four (stage IV, age > 60 years, large-cell transformation, and increased lactate dehydrogenase) were independent prognostic markers for a worse survival. Combining these four factors in a prognostic index model identified the following three risk groups across stages with significantly different 5-year survival rates: low risk (68%), intermediate risk (44%), and high risk (28%). Conclusion To our knowledge, this study includes the largest cohort of patients with advanced-stage MF/SS and identifies markers with independent prognostic value, which, used together in a prognostic index, may be useful to stratify advanced-stage patients.

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“…Patients with Sézary syndrome (SS), a rare erythrodermic and leukemic form of cutaneous T-cell lymphoma, have a poor prognosis with a 5-year overall survival (OS) of 20e42% and a median OS between 2.5 and 5.0 years (Agar et al, 2010;Bernengo et al, 1998;Diamandidou et al, 1999;Kim et al, 2003;Kubica et al, 2012;Talpur et al, 2012).…”

Section: To the Editormentioning

confidence: 99%

“…Prognostic factors associated with a worse survival reported in SS include advanced age (Agar et al, 2010;Diamandidou et al, 1999;Foulc et al, 2003;Kim et al, 2003;Kubica et al, 2012;Talpur et al, 2012), short duration of skin lesions before diagnosis of SS (Foulc et al, 2003), previous history of mycosis fungoides (Bernengo et al, 1998;Kubica et al, 2012), elevated serum lactate dehydrogenase levels (Agar et al, 2010;Bernengo et al, 1998;Diamandidou et al, 1999;Foulc et al, 2003;Kubica et al, 2012;Talpur et al, 2012), degree of nodal involvement (Diamandidou et al, 1999;Kim et al, 2003), and factors reflecting blood tumor burden, such as increased leukocyte counts (Bernengo et al, 1998;Talpur et al, 2012;Vidulich et al, 2009) or high Sézary cell counts (Bernengo et al, 1998).…”

Section: To the Editormentioning

confidence: 99%