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Robert Knobler - One of the best experts on this subject based on the ideXlab platform.
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characteristics associated with significantly worse quality of life in mycosis fungoides sezary syndrome from the prospective Cutaneous Lymphoma international prognostic index proclipi study
British Journal of Dermatology, 2020Co-Authors: Kevin Molloy, Robert Knobler, Constanze Jonak, F J S H Woeiajin, Emmanuella Guenova, A M Busschots, A Bervoets, Esther Hauben, Stefanie Porkert, Christina FassnachtAbstract:BACKGROUND: Mycosis fungoides (MF) and Sezary Syndrome (SS) are the most common Cutaneous T-cell Lymphomas. MF/SS is accompanied by considerable morbidity from pain, itching and disfigurement. AIM: To identify factors associated with poorer health-related quality of life (HRQoL) in patients newly diagnosed with MF/SS. METHODS: Patients enrolled into Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI; an international observational study in MF/SS) had their HRQoL assessed using the Skindex-29 questionnaire. Skindex-29 scores were analysed in relation to patient- and disease-specific characteristics. RESULTS: The study population consisted of 237 patients [60.3% male; median age 60 years, (interquartile range 49-70)], of whom 179 had early MF and 58 had advanced MF/SS. In univariate analysis, HRQoL, as measured by Skindex-29, was worse in women, SS, late-stage MF, those with elevated lactate dehydrogenase, alopecia, high modified Severity Weighted Assessment Tool and confluent erythema. Linear regression models only identified female gender (beta = 8.61; P = 0.003) and alopecia (beta = 9.71, P = 0.02) as independent predictors of worse global HRQoL. Item-level analysis showed that the severe impairment in symptoms [odds ratio (OR) 2.14, 95% confidence interval (CI) 1.19-3.89] and emotions (OR 1.88, 95% CI 1.09-3.27) subscale scores seen in women was caused by more burning/stinging, pruritus, irritation and greater feelings of depression, shame, embarrassment and annoyance with their diagnosis of MF/SS. CONCLUSIONS: HRQoL is significantly more impaired in newly diagnosed women with MF/SS and in those with alopecia. As Skindex-29 does not include existential questions on cancer, which may cause additional worry and distress, a comprehensive validated Cutaneous T-cell Lymphoma-specific questionnaire is urgently needed to more accurately assess disease-specific HRQoL in these patients. What's already known about this topic? Cross-sectional studies of mixed populations of known and newly diagnosed patients with mycosis fungoides (MF)/Sezary syndrome (SS) have shown significant impairment in health-related quality of life (HRQoL). Previous studies on assessing gender-specific differences in HRQoL in MF/SS are conflicting. More advanced-stage disease and pruritus is associated with poorer HRQoL in patients with MF/SS. What does this study add? This is the first prospective study to investigate HRQoL in a homogenous group of newly diagnosed patients with MF/SS. In patients newly diagnosed with MF/SS, HRQoL is worse in women and in those with alopecia and confluent erythema. MF/SS diagnosis has a multidimensional impact on patient HRQoL, including a large burden of Cutaneous symptoms, as well as a negative impact on emotional well-being.
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characteristics associated with significantly worse quality of life in mycosis fungoides sezary syndrome from the prospective Cutaneous Lymphoma international prognostic index proclipi study
British Journal of Dermatology, 2020Co-Authors: Kevin Molloy, Robert Knobler, Constanze Jonak, F J S H Woeiajin, Emmanuella Guenova, A M Busschots, A Bervoets, Esther Hauben, Stefanie Porkert, Christina FassnachtAbstract:Background Mycosis fungoides (MF) and Sezary Syndrome (SS) are the most common Cutaneous T-cell Lymphomas. MF/SS is accompanied by considerable morbidity from pain, itching and disfigurement. Aim To identify factors associated with poorer health-related quality of life (HRQoL) in patients newly diagnosed with MF/SS. Methods Patients enrolled into Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI; an international observational study in MF/SS) had their HRQoL assessed using the Skindex-29 questionnaire. Skindex-29 scores were analysed in relation to patient- and disease-specific characteristics. Results The study population consisted of 237 patients [60·3% male; median age 60 years, (interquartile range 49-70)], of whom 179 had early MF and 58 had advanced MF/SS. In univariate analysis, HRQoL, as measured by Skindex-29, was worse in women, SS, late-stage MF, those with elevated lactate dehydrogenase, alopecia, high modified Severity Weighted Assessment Tool and confluent erythema. Linear regression models only identified female gender (β = 8·61; P = 0·003) and alopecia (β = 9·71, P = 0·02) as independent predictors of worse global HRQoL. Item-level analysis showed that the severe impairment in symptoms [odds ratio (OR) 2·14, 95% confidence interval (CI) 1·19-3·89] and emotions (OR 1·88, 95% CI 1·09-3·27) subscale scores seen in women was caused by more burning/stinging, pruritus, irritation and greater feelings of depression, shame, embarrassment and annoyance with their diagnosis of MF/SS. Conclusions HRQoL is significantly more impaired in newly diagnosed women with MF/SS and in those with alopecia. As Skindex-29 does not include existential questions on cancer, which may cause additional worry and distress, a comprehensive validated Cutaneous T-cell Lymphoma-specific questionnaire is urgently needed to more accurately assess disease-specific HRQoL in these patients. What's already known about this topic? Cross-sectional studies of mixed populations of known and newly diagnosed patients with mycosis fungoides (MF)/Sezary syndrome (SS) have shown significant impairment in health-related quality of life (HRQoL). Previous studies on assessing gender-specific differences in HRQoL in MF/SS are conflicting. More advanced-stage disease and pruritus is associated with poorer HRQoL in patients with MF/SS. What does this study add? This is the first prospective study to investigate HRQoL in a homogenous group of newly diagnosed patients with MF/SS. In patients newly diagnosed with MF/SS, HRQoL is worse in women and in those with alopecia and confluent erythema. MF/SS diagnosis has a multidimensional impact on patient HRQoL, including a large burden of Cutaneous symptoms, as well as a negative impact on emotional well-being.
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blood classification and blood response criteria in mycosis fungoides and sezary syndrome using flow cytometry recommendations from the eortc Cutaneous Lymphoma task force
European Journal of Cancer, 2018Co-Authors: Julia Scarisbrick, Robert Knobler, Emmilia Hodak, Martine Bagot, René Stranzenbach, Rudolf Stadler, Evangelia Papadavid, Felicity Evison, Pablo L Ortizromero, Pietro QuaglinoAbstract:Abstract Our current mycosis fungoides (MF) and Sezary Syndrome (SS) staging system includes blood-classification from B0-B2 for patch/plaque/tumour or erythroderma based on manual Sezary counts but results from our EORTC survey confirm these are rarely performed in patch/plaque/tumour MF, and there is a trend towards using flow cytometry to measure blood-class. Accurately assigning blood-class effects overall stage and the ‘global response’ used to measure treatment responses in MF/SS and hence impacts management. The EORTC Cutaneous Lymphoma Task Force Committee have reviewed the literature and held a Workshop (June 2017) to agree a definition of blood-class according to flow cytometry. No large study comparing blood-class as defined by Sezary count with flow cytometry has been performed in MF/SS. The definition of blood-class by flow cytometry varies between publications. Low-level blood involvement occurs in patch/plaque/tumour much less than erythroderma (p For consistency flow, definition for blood-class must be an objective measurement. We propose absolute counts of either CD4+CD7-or CD4+CD26-where B0
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Blood classification and blood response criteria in mycosis fungoides and Sézary syndrome using flow cytometry: recommendations from the EORTC Cutaneous Lymphoma task force.
European journal of cancer (Oxford England : 1990), 2018Co-Authors: Julia Scarisbrick, Robert Knobler, Emmilia Hodak, Martine Bagot, René Stranzenbach, Rudolf Stadler, Pablo L. Ortiz-romero, Evangelia Papadavid, Felicity Evison, Pietro QuaglinoAbstract:Our current mycosis fungoides (MF) and Sézary Syndrome (SS) staging system includes blood-classification from B0-B2 for patch/plaque/tumour or erythroderma based on manual Sézary counts but results from our EORTC survey confirm these are rarely performed in patch/plaque/tumour MF, and there is a trend towards using flow cytometry to measure blood-class. Accurately assigning blood-class effects overall stage and the 'global response' used to measure treatment responses in MF/SS and hence impacts management. The EORTC Cutaneous Lymphoma Task Force Committee have reviewed the literature and held a Workshop (June 2017) to agree a definition of blood-class according to flow cytometry. No large study comparing blood-class as defined by Sézary count with flow cytometry has been performed in MF/SS. The definition of blood-class by flow cytometry varies between publications. Low-level blood involvement occurs in patch/plaque/tumour much less than erythroderma (p
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Major achievements of the EORTC Cutaneous Lymphoma Task Force (CLTF)
European Journal of Cancer Supplements, 2012Co-Authors: Sean Whittaker, Robert Knobler, Reinhard Dummer, Pablo Ortiz, Maarten H. Vermeer, Matthias Karrasch, Baktiar Hasan, Denis Lacombe, Werner Kempf, Martine BagotAbstract:Abstract This article describes the achievements of the Cutaneous LymphomaTask Force (CLTF) over the recent decade in their goal to optimize classification and response criteria and establish new treatment options for patients suffering from Cutaneous T-cell Lymphomas (CTCL). Collaborative work with the International Society of Cutaneous Lymphoma (ISCL) and the United States Cutaneous Lymphoma Consortium (USCLC) has led to publication of pivotal manuscripts proposing revised staging proposals for Mycosis fungoides (MF)/Sezary syndrome (SS) and also non MF/SS primary Cutaneous Lymphomas as well as the recent publication of a proposal for defining endpoints in MF/SS.
Elise A Olsen - One of the best experts on this subject based on the ideXlab platform.
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guidelines for phototherapy of mycosis fungoides and sezary syndrome a consensus statement of the united states Cutaneous Lymphoma consortium
Journal of The American Academy of Dermatology, 2016Co-Authors: Elise A Olsen, Joi B. Carter, Emilia Hodak, Thomas F Anderson, Marsha Henderson, Kevin D Cooper, Henry W LimAbstract:Background Ultraviolet light (UVL) is a long established treatment for mycosis fungoides (MF) and Sezary syndrome (SS), subtypes of Cutaneous T-cell Lymphoma (CTCL). Treatments have traditionally included broadband, narrowband ultraviolet B light (UVB) and psoralen plus ultraviolet A light photochemotherapy (PUVA), but more recently, treatment options have expanded to include UVA1 and excimer laser. UVL is used either as monotherapy or as an adjuvant to systemic therapy, demonstrating efficacy in many cases that equal or surpass systemic medications. Despite its utility and duration of use, the current practice of using UVL guidelines for psoriasis to treat patients with MF/SS is problematic because the goals of prolonging survival and preventing disease progression are unique to CTCL compared to psoriasis. Objectives We sought to develop separate guidelines for phototherapy for MF/SS for both clinical practice and for clinical trials. Methods Literature review and Cutaneous Lymphoma expert consensus group recommendations. Results This paper reviews the published literature for UVB and UVA/PUVA in MF/SS and suggests practical standardized guidelines for their use. Limitations New standardization of phototherapy. Conclusions These guidelines should allow the comparison of results with phototherapy in MF/SS across different stages of patients, centers, and in combination with other agents in practice and in clinical trials.
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sezary syndrome immunopathogenesis literature review of therapeutic options and recommendations for therapy by the united states Cutaneous Lymphoma consortium usclc
Journal of The American Academy of Dermatology, 2011Co-Authors: Elise A Olsen, Gary S Wood, Marie-france Demierre, Youn H. Kim, Alain H Rook, John A Zic, Pierluigi Porcu, Christiane Querfeld, Mark R Pittelkow, Lynn D WilsonAbstract:Sezary syndrome (SS) has a poor prognosis and few guidelines for optimizing therapy. The US Cutaneous Lymphoma Consortium, to improve clinical care of patients with SS and encourage controlled clinical trials of promising treatments, undertook a review of the published literature on therapeutic options for SS. An overview of the immunopathogenesis and standardized review of potential current treatment options for SS including metabolism, mechanism of action, overall efficacy in mycosis fungoides and SS, and common or concerning adverse effects is first discussed. The specific efficacy of each treatment for SS, both as monotherapy and combination therapy, is then reported using standardized criteria for both SS and response to therapy with the type of study defined by a modification of the US Preventive Services guidelines for evidence-based medicine. Finally, guidelines for the treatment of SS and suggestions for adjuvant treatment are noted.
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revisions to the staging and classification of mycosis fungoides and sezary syndrome a proposal of the international society for Cutaneous Lymphomas iscl and the Cutaneous Lymphoma task force of the european organization of research and treatment of
Blood, 2007Co-Authors: Elise A Olsen, Stanford I Lamberg, Nicola Pimpinelli, Herschel S Zackheim, Robert Knobler, Rein Willemze, Madeleine Duvic, Teresa Estrach, Eric C Vonderheid, Gary S WoodAbstract:The ISCL/EORTC recommends revisions to the Mycosis Fungoides Cooperative Group classification and staging system for Cutaneous T-cell Lymphoma (CTCL). These revisions are made to incorporate advances related to tumor cell biology and diagnostic techniques as pertains to mycosis fungoides (MF) and Sezary syndrome (SS) since the 1979 publication of the original guidelines, to clarify certain variables that currently impede effective interinstitution and interinvestigator communication and/or the development of standardized clinical trials in MF and SS, and to provide a platform for tracking other variables of potential prognostic significance. Moreover, given the difference in prognosis and clinical characteristics of the non-MF/non-SS subtypes of Cutaneous Lymphoma, this revision pertains specifically to MF and SS. The evidence supporting the revisions is discussed as well as recommendations for evaluation and staging procedures based on these revisions.
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revisions to the staging and classification of mycosis fungoides and sezary syndrome a proposal of the international society for Cutaneous Lymphomas iscl and the Cutaneous Lymphoma task force of the european organization of research and treatment of
Blood, 2007Co-Authors: Elise A Olsen, Stanford I Lamberg, Nicola Pimpinelli, Herschel S Zackheim, Robert Knobler, Rein Willemze, Madeleine Duvic, Teresa Estrach, Eric C Vonderheid, Gary S WoodAbstract:The ISCL/EORTC recommends revisions to the Mycosis Fungoides Cooperative Group classification and staging system for Cutaneous T-cell Lymphoma (CTCL). These revisions are made to incorporate advances related to tumor cell biology and diagnostic techniques as pertains to mycosis fungoides (MF) and Sezary syndrome (SS) since the 1979 publication of the original guidelines, to clarify certain variables that currently impede effective interinstitution and interinvestigator communication and/or the development of standardized clinical trials in MF and SS, and to provide a platform for tracking other variables of potential prognostic significance. Moreover, given the difference in prognosis and clinical characteristics of the non-MF/non-SS subtypes of Cutaneous Lymphoma, this revision pertains specifically to MF and SS. The evidence supporting the revisions is discussed as well as recommendations for evaluation and staging procedures based on these revisions.
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tnm classification system for primary Cutaneous Lymphomas other than mycosis fungoides and sezary syndrome a proposal of the international society for Cutaneous Lymphomas iscl and the Cutaneous Lymphoma task force of the european organization of rese
Blood, 2007Co-Authors: Youn H. Kim, Elise A Olsen, Nicola Pimpinelli, Rein Willemze, Reinhard Dummer, Sean Whittaker, Annamari Ranki, Richard T HoppeAbstract:Currently availabel staging systems for non-Hodgkin Lymphomas are not useful for clinical staging classification of most primary Cutaneous Lymphomas. The tumor, node, metastases (TNM) system used for mycosis fungoides (MF) and Sezary syndrome (SS) is not appropriate for other primary Cutaneous Lymphomas. A usable, unified staging system would improve the communication about the state of disease, selection of appropriate management, standardization of enrollment/response criteria in clinical trials, and collection/analysis of prospective survival data. Toward this goal, during the recent meetings of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC), the representatives have established a consensus proposal of a TNM classification system applicable for all primary Cutaneous Lymphomas other than MF and SS. Due to the clinical and pathologic heterogeneity of the Cutaneous Lymphomas, the currently proposed TNM system is meant to be primarily an anatomic documentation of disease extent and not to be used as a prognostic guide.
Nicola Pimpinelli - One of the best experts on this subject based on the ideXlab platform.
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standardization of regimens in narrowband uvb and puva in early stage mycosis fungoides position paper from the italian task force for Cutaneous Lymphomas
Journal of The European Academy of Dermatology and Venereology, 2018Co-Authors: Vieri Grandi, Pietro Quaglino, E Berti, Paolo Fava, Serena Rupoli, Alberti S Violetti, Laura Canafoglia, Nicola PimpinelliAbstract:UV-based (PUVA and narrowband UVB) phototherapy is broadly and commonly used in the treatment of Cutaneous T-cell Lymphomas (CTCL), yet unfortunately, the evidence for the efficacy of these treatments is based only on case series or prospective but non-randomized studies. Therefore, no internationally approved guidelines exist and no standardization of schedules has been proposed. Recently, consensus guidelines have been published by the United States Cutaneous Lymphoma Consortium. The aim of this study was to review the biological and clinical evidences on PUVA and NB-UVB in CTCL and to critically evaluate acceptability and feasibility of these guidelines in the real-life setting from the perspective of the Cutaneous Lymphoma Task Force of the Italian Lymphoma Foundation (Fondazione Italiana Linfomi, FIL).
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european organization for research and treatment of cancer and international society for Cutaneous Lymphoma consensus recommendations for the management of Cutaneous b cell Lymphomas
Blood, 2008Co-Authors: Nancy J Senff, Madeleine Duvic, Lorenzo Cerroni, Youn H. Kim, Reinhard Dummer, E M Noordijk, M Bagot, E Berti, Richard T Hoppe, Nicola PimpinelliAbstract:Primary Cutaneous B-cell Lymphomas (CBCL) represent approximately 20% to 25% of all primary Cutaneous Lymphomas. With the advent of the World Health Organization-European Organization for Research and Treatment of Cancer (EORTC) Consensus Classification for Cutaneous Lymphomas in 2005, uniform terminology and classification for this rare group of neoplasms were introduced. However, staging procedures and treatment strategies still vary between different Cutaneous Lymphoma centers, which may be because consensus recommendations for the management of CBCL have never been published. Based on an extensive literature search and discussions within the EORTC Cutaneous Lymphoma Group and the International Society for Cutaneous Lymphomas, the present report aims to provide uniform recommendations for the management of the 3 main groups of CBCL. Because no systematic reviews or (randomized) controlled trials were available, these recommendations are mainly based on retrospective studies and small cohort studies. Despite these limitations, there was consensus among the members of the multidisciplinary expert panel that these recommendations reflect the state-of-the-art management as currently practiced in major Cutaneous Lymphoma centers. They may therefore contribute to uniform staging and treatment and form the basis for future clinical trials in patients with a CBCL.
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revisions to the staging and classification of mycosis fungoides and sezary syndrome a proposal of the international society for Cutaneous Lymphomas iscl and the Cutaneous Lymphoma task force of the european organization of research and treatment of
Blood, 2007Co-Authors: Elise A Olsen, Stanford I Lamberg, Nicola Pimpinelli, Herschel S Zackheim, Robert Knobler, Rein Willemze, Madeleine Duvic, Teresa Estrach, Eric C Vonderheid, Gary S WoodAbstract:The ISCL/EORTC recommends revisions to the Mycosis Fungoides Cooperative Group classification and staging system for Cutaneous T-cell Lymphoma (CTCL). These revisions are made to incorporate advances related to tumor cell biology and diagnostic techniques as pertains to mycosis fungoides (MF) and Sezary syndrome (SS) since the 1979 publication of the original guidelines, to clarify certain variables that currently impede effective interinstitution and interinvestigator communication and/or the development of standardized clinical trials in MF and SS, and to provide a platform for tracking other variables of potential prognostic significance. Moreover, given the difference in prognosis and clinical characteristics of the non-MF/non-SS subtypes of Cutaneous Lymphoma, this revision pertains specifically to MF and SS. The evidence supporting the revisions is discussed as well as recommendations for evaluation and staging procedures based on these revisions.
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revisions to the staging and classification of mycosis fungoides and sezary syndrome a proposal of the international society for Cutaneous Lymphomas iscl and the Cutaneous Lymphoma task force of the european organization of research and treatment of
Blood, 2007Co-Authors: Elise A Olsen, Stanford I Lamberg, Nicola Pimpinelli, Herschel S Zackheim, Robert Knobler, Rein Willemze, Madeleine Duvic, Teresa Estrach, Eric C Vonderheid, Gary S WoodAbstract:The ISCL/EORTC recommends revisions to the Mycosis Fungoides Cooperative Group classification and staging system for Cutaneous T-cell Lymphoma (CTCL). These revisions are made to incorporate advances related to tumor cell biology and diagnostic techniques as pertains to mycosis fungoides (MF) and Sezary syndrome (SS) since the 1979 publication of the original guidelines, to clarify certain variables that currently impede effective interinstitution and interinvestigator communication and/or the development of standardized clinical trials in MF and SS, and to provide a platform for tracking other variables of potential prognostic significance. Moreover, given the difference in prognosis and clinical characteristics of the non-MF/non-SS subtypes of Cutaneous Lymphoma, this revision pertains specifically to MF and SS. The evidence supporting the revisions is discussed as well as recommendations for evaluation and staging procedures based on these revisions.
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tnm classification system for primary Cutaneous Lymphomas other than mycosis fungoides and sezary syndrome a proposal of the international society for Cutaneous Lymphomas iscl and the Cutaneous Lymphoma task force of the european organization of rese
Blood, 2007Co-Authors: Youn H. Kim, Elise A Olsen, Nicola Pimpinelli, Rein Willemze, Reinhard Dummer, Sean Whittaker, Annamari Ranki, Richard T HoppeAbstract:Currently availabel staging systems for non-Hodgkin Lymphomas are not useful for clinical staging classification of most primary Cutaneous Lymphomas. The tumor, node, metastases (TNM) system used for mycosis fungoides (MF) and Sezary syndrome (SS) is not appropriate for other primary Cutaneous Lymphomas. A usable, unified staging system would improve the communication about the state of disease, selection of appropriate management, standardization of enrollment/response criteria in clinical trials, and collection/analysis of prospective survival data. Toward this goal, during the recent meetings of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC), the representatives have established a consensus proposal of a TNM classification system applicable for all primary Cutaneous Lymphomas other than MF and SS. Due to the clinical and pathologic heterogeneity of the Cutaneous Lymphomas, the currently proposed TNM system is meant to be primarily an anatomic documentation of disease extent and not to be used as a prognostic guide.
Rein Willemze - One of the best experts on this subject based on the ideXlab platform.
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mimics of Cutaneous Lymphoma report of the 2011 society for hematopathology european association for haematopathology workshop
American Journal of Clinical Pathology, 2013Co-Authors: George P Sarantopoulos, Rein Willemze, Steven H Swerdlow, Marsha C Kinney, Beth Palla, Jonathan W Said, Scott W BinderAbstract:The Society for Hematopathology and European Association for Haematopathology workshop, from October 27 to 29, 2011, in Los Angeles, CA, exhibited many exemplary skin biopsy specimens with interesting inflammatory changes mimicking features of Cutaneous Lymphoma. This article reviews features observed in Cutaneous lymphoid hyperplasia, Cutaneous drug reactions, lupus-associated panniculitis, pityriasis lichenoides, hypereosinophilic syndrome, histiocytic necrotizing lymphadenitis, traumatic ulcerative granuloma with stromal eosinophils, and pigmented purpuric dermatosis, as well as a brief review of the pertinent literature and discussion of submitted conference cases. For the pathologist, it is important to be aware of diagnostic pitfalls as well as the limitations of ancillary testing (eg, clonality studies). Finally, correlation with total clinical information, good communication with clinical colleagues, close clinical follow-up with rebiopsy, and prudent use of laboratory studies are vital and will likely offer the best path toward a correct diagnosis.
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Mimics of Cutaneous Lymphoma report of the 2011 society for hematopathology/European association for haematopathology workshop
American Journal of Clinical Pathology, 2013Co-Authors: George P Sarantopoulos, Rein Willemze, Marsha C Kinney, Beth Palla, Steven M. Swerdlow, Jonathan W Said, Scott W BinderAbstract:The Society for Hematopathology and European Association for Haematopathology workshop, from October 27 to 29, 2011, in Los Angeles, CA, exhibited many exemplary skin biopsy specimens with interesting inflammatory changes mimicking features of Cutaneous Lymphoma. This article reviews features observed in Cutaneous lymphoid hyperplasia, Cutaneous drug reactions, lupus-associated panniculitis, pityriasis lichenoides, hypereosinophilic syndrome, histiocytic necrotizing lymphadenitis, traumatic ulcerative granuloma with stromal eosinophils, and pigmented purpuric dermatosis, as well as a brief review of the pertinent literature and discussion of submitted conference cases. For the pathologist, it is important to be aware of diagnostic pitfalls as well as the limitations of ancillary testing (eg, clonality studies). Finally, correlation with total clinical information, good communication with clinical colleagues, close clinical follow-up with rebiopsy, and prudent use of laboratory studies are vital and will likely offer the best path toward a correct diagnosis.
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mimics of Cutaneous Lymphoma report of the 2011 society for hematopathology european association for haematopathology workshop
American Journal of Clinical Pathology, 2013Co-Authors: George P Sarantopoulos, Rein Willemze, Marsha C Kinney, Beth Palla, Steven M. Swerdlow, Jonathan W Said, Scott W BinderAbstract:The Society for Hematopathology and European Association for Haematopathology workshop, from October 27 to 29, 2011, in Los Angeles, CA, exhibited many exemplary skin biopsy specimens with interesting inflammatory changes mimicking features of Cutaneous Lymphoma. This article reviews features observed in Cutaneous lymphoid hyperplasia, Cutaneous drug reactions, lupus-associated panniculitis, pityriasis lichenoides, hypereosinophilic syndrome, histiocytic necrotizing lymphadenitis, traumatic ulcerative granuloma with stromal eosinophils, and pigmented purpuric dermatosis, as well as a brief review of the pertinent literature and discussion of submitted conference cases. For the pathologist, it is important to be aware of diagnostic pitfalls as well as the limitations of ancillary testing (eg, clonality studies). Finally, correlation with total clinical information, good communication with clinical colleagues, close clinical follow-up with rebiopsy, and prudent use of laboratory studies are vital and will likely offer the best path toward a correct diagnosis.
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subCutaneous panniculitis like t cell Lymphoma definition classification and prognostic factors an eortc Cutaneous Lymphoma group study of 83 cases
Blood, 2008Co-Authors: Rein Willemze, Lorenzo Cerroni, Marco Santucci, E Berti, Patty M Jansen, Chalid Assaf, Marijke Canningavan R Dijk, Agnes Carlotti, Marielouise Geerts, Sonja HahtolaAbstract:In the WHO classification, subCutaneous panniculitis-like T-cell Lymphoma (SPTL) is defined as a distinct type of T-cell Lymphoma with an aggressive clinical behavior. Recent studies suggest that distinction should be made between SPTL with an α/β T-cell phenotype (SPTL-AB) and SPTL with a γδ T-cell phenotype (SPTL-GD), but studies are limited. To better define their clinicopathologic features, immunophenotype, treatment, and survival, 63 SPTL-ABs and 20 SPTL-GDs were studied at a workshop of the EORTC Cutaneous Lymphoma Group. SPTL-ABs were generally confined to the subcutis, had a CD4−, CD8+, CD56−, βF1+ phenotype, were uncommonly associated with a hemophagocytic syndrome (HPS; 17%), and had a favorable prognosis (5-year overall survival [OS]: 82%). SPTL-AB patients without HPS had a significantly better survival than patients with HPS (5-year OS: 91% vs 46%; P < .001). SPTL-GDs often showed (epi)dermal involvement and/or ulceration, a CD4−, CD8−, CD56+/−, βF1− T-cell phenotype, and poor prognosis (5-year OS: 11%), irrespective of the presence of HPS or type of treatment. These results indicate that SPTL-AB and SPTL-GD are distinct entities, and justify that the term SPTL should further be used only for SPTL-AB. SPTL-ABs without associated HPS have an excellent prognosis, and multiagent chemotherapy as first choice of treatment should be questioned.
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subCutaneous panniculitis like t cell Lymphoma definition classification and prognostic factors an eortc Cutaneous Lymphoma group study of 83 cases
Blood, 2008Co-Authors: Rein Willemze, Lorenzo Cerroni, Marco Santucci, E Berti, Patty M Jansen, Chalid Assaf, Marijke Canningavan R Dijk, Agnes Carlotti, Marielouise Geerts, Sonja HahtolaAbstract:In the WHO classification, subCutaneous panniculitis-like T-cell Lymphoma (SPTL) is defined as a distinct type of T-cell Lymphoma with an aggressive clinical behavior. Recent studies suggest that distinction should be made between SPTL with an alpha/beta T-cell phenotype (SPTL-AB) and SPTL with a gammadelta T-cell phenotype (SPTL-GD), but studies are limited. To better define their clinicopathologic features, immunophenotype, treatment, and survival, 63 SPTL-ABs and 20 SPTL-GDs were studied at a workshop of the EORTC Cutaneous Lymphoma Group. SPTL-ABs were generally confined to the subcutis, had a CD4-, CD8+, CD56-, betaF1+ phenotype, were uncommonly associated with a hemophagocytic syndrome (HPS; 17%), and had a favorable prognosis (5-year overall survival [OS]: 82%). SPTL-AB patients without HPS had a significantly better survival than patients with HPS (5-year OS: 91% vs 46%; P<.001). SPTL-GDs often showed (epi)dermal involvement and/or ulceration, a CD4-, CD8-, CD56+/-, betaF1- T-cell phenotype, and poor prognosis (5-year OS: 11%), irrespective of the presence of HPS or type of treatment. These results indicate that SPTL-AB and SPTL-GD are distinct entities, and justify that the term SPTL should further be used only for SPTL-AB. SPTL-ABs without associated HPS have an excellent prognosis, and multiagent chemotherapy as first choice of treatment should be questioned.
Lynn D Wilson - One of the best experts on this subject based on the ideXlab platform.
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sezary syndrome immunopathogenesis literature review of therapeutic options and recommendations for therapy by the united states Cutaneous Lymphoma consortium usclc
Journal of The American Academy of Dermatology, 2011Co-Authors: Elise A Olsen, Gary S Wood, Marie-france Demierre, Youn H. Kim, Alain H Rook, John A Zic, Pierluigi Porcu, Christiane Querfeld, Mark R Pittelkow, Lynn D WilsonAbstract:Sezary syndrome (SS) has a poor prognosis and few guidelines for optimizing therapy. The US Cutaneous Lymphoma Consortium, to improve clinical care of patients with SS and encourage controlled clinical trials of promising treatments, undertook a review of the published literature on therapeutic options for SS. An overview of the immunopathogenesis and standardized review of potential current treatment options for SS including metabolism, mechanism of action, overall efficacy in mycosis fungoides and SS, and common or concerning adverse effects is first discussed. The specific efficacy of each treatment for SS, both as monotherapy and combination therapy, is then reported using standardized criteria for both SS and response to therapy with the type of study defined by a modification of the US Preventive Services guidelines for evidence-based medicine. Finally, guidelines for the treatment of SS and suggestions for adjuvant treatment are noted.
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total skin electron radiation in the management of mycosis fungoides consensus of the european organization for research and treatment of cancer eortc Cutaneous Lymphoma project group
Journal of The American Academy of Dermatology, 2002Co-Authors: Glenn W Jones, Rein Willemze, Margaret Spittle, Lynn D Wilson, Barry M Kacinski, Gerda Hohenberg, Leonore Handlzeller, Franz Trautinger, Robert KnoblerAbstract:Radiotherapy has been successfully implemented in the treatment of mycosis fungoides (MF) for almost a century. With the development of the modern linear accelerator, it has become possible to treat extended areas of the skin with accelerated electrons. Total skin electron beam radiation (TSEB) has been in use for several decades, and a number of technical modifications have been made with the goals of optimizing dose distribution and improving clinical outcome. Emerging evidence from recent studies suggests an association between TSEB techniques and efficacy in the treatment of MF. Based on this evidence, the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group, in association with experts from radiotherapy centers in North America, has reached a consensus on acceptable methods and clinical indications for TSEB in the treatment of MF. The aims of this report are to enhance accessibility of this highly efficacious treatment modality to patients with MF and to provide a point of reference for further clinical research.
