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BACKGROUND: Treatment choices for patients with advanced-stage mycosis fungoides (MF) or Sézary syndrome (SS) who have failed first-line systemic therapies can be challenging, as several options are available. However, most evidence is based on observational and early phase studies due to the rarity of the disease. Mogamulizumab has recently been approved for the treatment of adult patients with MF or SS who have received at least one prior systemic therapy; it has a good tolerability profile prompting its use in combination with other agents. This article aims at describing the role of the concomitant use of bexarotene with mogamulizumab in this setting. CASES PRESENTATION: To add information in the field, we describe our experience with four patients with MF/SS who failed first- and second-line treatments and started the combination mogamulizumab in addition to bexarotene. The combination of bexarotene with mogamulizumab in patients with advanced MF/SS after the failure of bexarotene alone obtained a response in all the four patients observed. The response was maintained longer than expected. CONCLUSIONS: The combination is promising and deserves further study. [ABSTRACT FROM AUTHOR]

Primary cutaneous T-cell lymphomas (CTCLs) other than mycosis fungoides (MF) and Sézary syndrome (SS) encompass a heterogenous group of non-Hodgkin lymphomas with variable clinical courses, prognoses, and management approaches. Given the morphologic and histologic overlap among the CTCL subtypes and other T-cell lymphomas with cutaneous manifestations, thorough evaluation with clinicopathologic correlation and exclusion of systemic involvement are essential prior to initiating therapy. Staging and treatment recommendations vary, depending on the subtype, clinical behavior, and treatment response. Generally, for subtypes in which staging is recommended, Ann Arbor or tumor, node, metastasis staging specific to CTCL other than MF or SS are used. For many subtypes, there is no standard treatment to date. Available recommended treatments range widely, from no active or minimal intervention with skin-directed therapy to aggressive systemic therapies that include multi-agent chemotherapy with consideration for hematopoietic stem cell transplant. Emerging targeted therapies, such as brentuximab, a chimeric antibody targeting CD30, show promise in altering the disease course of non-MF/SS CTCLs. [ABSTRACT FROM AUTHOR]

Primary cutaneous T-cell lymphomas (CTCLs) are defined as lymphomas with a T-cell phenotype that present in the skin without evidence of systemic or extracutaneous disease at initial presentation. CTCLs other than mycosis fungoides and Sézary syndrome (SS) account for approximately one third of CTCLs and encompass a heterogenous group of non-Hodgkin lymphomas, ranging from indolent lymphoproliferative disorders to aggressive malignancies with a poor prognosis. The spectrum of CTCLs continues to broaden as new provisional entities are classified. Given the morphologic and histologic overlap among CTCLs and other diagnoses, a thorough clinical history, physical evaluation, and clinicopathologic correlation are essential in the work up and diagnosis of these rare entities. This article will summarize the epidemiologic, clinical, pathologic, and diagnostic features of CTCLs other than mycosis fungoides and SS. [ABSTRACT FROM AUTHOR]

Adoptive cell therapy (ACT) is an emerging powerful cancer immunotherapy, which includes a complex process of genetic modification, stimulation and expansion. During these in vitro or ex vivo manipulation, sensitive cells are inescapability subjected to harmful external stimuli. Although a variety of cytoprotection strategies have been developed, their application on ACT remains challenging. Herein, a DNA network is constructed on cell surface by rolling circle amplification (RCA), and T cell-targeted trivalent tetrahedral DNA nanostructure is used as a rigid scaffold to achieve high-efficient and selective coating for T cells. The cytoprotective DNA network on T-cell surface makes them aggregate over time to form cell clusters, which exhibit more resistance to external stimuli and enhanced activities in human peripheral blood mononuclear cells and liver cancer organoid killing model. Overall, this work provides a novel strategy for in vitro T cell-selective protection, which has a great potential for application in ACT. T cell-selective coating and protection strategy with a flexible DNA network was formed by rolling circle amplification, which promotes T-cell aggregation and activation for anti-tumor therapy. [Display omitted] [ABSTRACT FROM AUTHOR]

Cutaneous T cell lymphomas (CTCLs) are malignancies of skin-trafficking T cells. Patients with advanced CTCL manifest immune dysfunction that predisposes to infection and suppresses the antitumor immune response. Therapies that stimulate immunity have produced superior progression-free survival compared with conventional chemotherapy, reinforcing the importance of addressing the immune deficient state in the care of patients with CTCL. Recent research has better defined the pathogenesis of these immune deficits, explaining the mechanisms of disease progression and revealing potential therapeutic targets. The features of the malignant cell in mycosis fungoides and Sézary syndrome are now significantly better understood, including the T helper 2 cell phenotype, regulatory T cell cytokine production, immune checkpoint molecule expression, chemokine receptors, and interactions with the microenvironment. The updated model of CTCL immunopathogenesis provides understanding into clinical progression and therapeutic response. [ABSTRACT FROM AUTHOR]

In the past few decades, immunotherapy has emerged as an effective therapeutic option for patients with cutaneous T cell lymphoma (CTCL). CTCL is characterized by progressive impairment of multiple arms of the immune system. Immunotherapy targets these deficits to stimulate a more robust antitumor response, thereby both clearing the malignant T cells and repairing the immune dysfunction. By potentiating rather than suppressing the immune system, immunotherapy can result in longer treatment responses than alternatives such as chemotherapy. In recent years, advances in our understanding of the pathogenesis of CTCL have led to the development of several new agents with promising efficacy profiles. The second article in this continuing medical education series describes the current immunotherapeutic options for treatment of CTCL, with a focus on how they interact with the immune system and their treatment outcomes in case studies and clinical trials. We will discuss established CTCL immunotherapies, such as interferons, photopheresis, and retinoids; emerging therapies, such as interleukin-12 and Toll-like receptor agonists; and new approaches to targeting tumor antigens and checkpoint molecules, such as mogamulizumab, anti-programmed cell death protein 1, anti-CD47, and chimeric antigen receptor T cell therapy. We also describe the principles of multimodality immunotherapy and the use of total skin electron beam therapy in such regimens. [ABSTRACT FROM AUTHOR]

The aim of this study was to assess treatment modalities, treatment response, toxicity profile, disease progression and outcomes in 14 patients with a confirmed diagnosis of primary cutaneous T-cell lymphoma (PCTCL) treated with total skin electron beam therapy (TSEBT). Primary cutaneous lymphomas (PCLs) are extranodal non-Hodgkin lymphomas originating in the skin without evidence of extracutaneous disease at diagnosis. Despite advances in systemic and local therapy options, the management of advanced stages remains mostly palliative. This is a retrospective study of patients with PCTCL, diagnosed and treated in a reference center in Mexico City, analyzing treatment modalities, response to treatment, long-term outcome, and mortality. Eight males (57%) and 6 (43%) females were identified. Most patients were stage IVA (n = 5, 36%) followed by stage IB and IIB (28.5% and 21.4%, respectively). Eleven patients received the low-dose RT scheme (12 Gy), 1 patient, the intermediate-dose RT scheme (24 Gy), and 2 patients, the conventional-dose RT scheme (36 Gy). Mean follow-up time was 4.6 years. At first follow-up examination, 6–8 weeks after radiotherapy, the overall response rate (ORR) for the cohort was 85%. The median PFS for the whole cohort was 6 months. This study reinforces the role of TSEBT when compared with other treatment modalities and novel agents. Low-dose TSEBT is now widely used because of the opportunity for retreatment. [ABSTRACT FROM AUTHOR]

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common types of cutaneous lymphoma, accounting for approximately 60% of cutaneous T-cell lymphomas. Diagnosis requires correlation of clinical, histologic, and molecular features. A multitude of factors have been linked to the aetiopathogenesis, however, none have been definitively proven. Erythrodermic MF (E-MF) and SS share overlapping clinical features, such as erythroderma, but are differentiated on the degree of malignant blood involvement. While related, they are considered to be two distinct entities originating from different memory T cell subsets. Differential expression of PD-1 and KIR3DL2 may represent a tool for distinguishing MF and SS, as well as a means of monitoring treatment response. Treatment of E-MF/SS is guided by disease burden, patients' ages and comorbidities, and effect on quality of life. Current treatment options include biologic, targeted, immunologic, and investigational therapies that can provide long term response with minimal side effects. Currently, allogeneic stem cell transplantation is the only potential curative treatment. [ABSTRACT FROM AUTHOR]

Background: Classic Sézary syndrome (SS) is defined by erythroderma, generalized lymphadenopathy, and leukemic blood involvement. Clinical observations suggest that SS begins as a nonerythrodermic disease.Objective: To describe the early clinical characteristics of patients with SS.Methods: A retrospective, multicenter chart review was performed for 263 confirmed cases of SS diagnosed during 1976-2015.Results: Erythroderma was the earliest recorded skin sign of SS in only 25.5% of cases, although most patients (86.3%) eventually developed erythroderma. In patients without erythroderma during their initial visit, the first cutaneous signs of SS were nonspecific dermatitis (49%), atopic dermatitis-like eruption (4.9%), or patches and plaques of mycosis fungoides (10.6%). The mean diagnostic delay was 4.2 years overall, 2.2 years for cases involving erythroderma at the initial presentation, and 5.0 years for cases not involving erythroderma at the initial presentation.Limitations: This study is retrospective.Conclusion: Erythroderma is uncommon as an initial sign of SS. Early SS should be considered in cases of nonerythrodermic dermatitis that is refractory to conventional treatments. In these cases, examination of the blood by PCR for monoclonal T-cell receptor rearrangement and by flow cytometry to identify an expanded or aberrant T-cell population should be considered. [ABSTRACT FROM AUTHOR]

Background: Sézary syndrome (SS) is characterized by erythroderma with leukemic involvement. In atypical SS, leukemic involvement is present without erythroderma. Little is known about the presentation, prognosis, and outcome in these patients.Objective: We sought to describe our experience with patients with SS without erythroderma.Methods: We retrospectively identified patients with SS, but without erythroderma, at Mayo Clinic from 1976 to 2010. Patients all met criteria for high blood tumor burden. Their clinical characteristics, disease course, and prognosis were reviewed and compared with a previously described cohort of 176 patients with SS from this institution.Results: Among 16 patients identified, all had cutaneous findings consistent with cutaneous T-cell lymphoma, most commonly erythematous patches (n = 6) and plaques (n = 12). All 16 patients were deceased at the time of the study. Median time from diagnosis of SS without erythroderma to the date of death was 3.6 years (range, 0.5-8.7 years). Survival was not different between patients with SS with and without erythroderma (hazard ratio 1.58; 95% confidence interval 0.94-2.66; P = .08).Limitations: This was a single-institution retrospective study.Conclusion: Leukemic involvement may confer shortened survival in patients with SS, because the presence of erythroderma did not affect survival. These atypical cases could potentially be more accurately described using the TNMB classification. [ABSTRACT FROM AUTHOR]

Background: Accurate quantification of malignant cells in the peripheral blood of patients with cutaneous T-cell lymphoma is important for early detection, prognosis, and monitoring disease burden.Objective: We sought to determine the spectrum of current clinical practices; critically evaluate elements of current International Society for Cutaneous Lymphomas (ISCL) B1 and B2 staging criteria; and assess the potential role of T-cell receptor-Vβ analysis by flow cytometry.Methods: We assessed current clinical practices by survey, and performed a retrospective analysis of 161 patients evaluated at Yale (2011-2014) to compare the sensitivity, specificity, positive predictive value, and negative predictive value of parameters for ISCL B2 staging.Results: There was heterogeneity in clinical practices among institutions. ISCL B1 criteria did not capture 5 Yale cohort cases with immunophenotypic abnormalities that later progressed. T-cell receptor-Vβ testing was more specific than polymerase chain reaction and aided diagnosis in detecting clonality, but was of limited benefit in quantification of tumor burden.Limitations: Because of limited follow-up involving a single center, further investigation will be necessary to conclude whether our proposed diagnostic algorithm is of general clinical benefit.Conclusion: We propose further study of modified B1 criteria: CD4/CD8 ratio 5 or greater, %CD4(+) CD26(-) 20% or greater, or %CD4(+) CD7(-) 20% or greater, with evidence of clonality. T-cell receptor-Vβ testing should be considered in future diagnostic and staging algorithms. [ABSTRACT FROM AUTHOR]

Background: Mycosis fungoides is the most common type of cutaneous T-cell lymphoma (CTCL), yet is frequently misdiagnosed and perplexing to those who may not be familiar with the disease process and management. Therefore, nursing involvement in the interdisciplinary referral process from a generalist to a specialist is key to improving patient outcomes in the early detection of mycosis fungoides. Purpose of Study/Inquiry: This manuscript elucidates the nursing role in the referral process of patients with CTCL, the importance of clinical grasp in the interdisciplinary approach for referrals, and factors to consider when customizing patient care plans. Methodology/Methods and Analytical Approach: In effort to elucidate the nursing role in the obscure referral process in this unique patient population, an evidencebased approach is utilized to create a standardized method of care coordination to improve patient outcomes. Harvesting this method improves clinical care coordination and can result in a powerful tool to streamline the referral process. Findings/Implications: An evidence-based standardized algorithm has been created for the nurse in the generalist office referring to patients with cutaneous lymphoma to dermatology specialists. Furthermore, this manuscript will strengthen nursing clinical knowledge, define the referral process, and improve patient outcomes in the transfer of care for patients with CTCL. [ABSTRACT FROM AUTHOR]

Chronic pruritic dermatitis with or without accompanying peripheral eosinophilia can be caused by a vast array of underlying disorders broadly classified as allergic/immunologic, infectious, or neoplastic. An organized and thorough work up is crucial in order to arrive at a definitive diagnosis enabling appropriate treatment. We present the case of a 42-year-old man with a history of chronic pruritic dermatitis and peripheral eosinophilia in a patient-oriented, problem-solving format including the clinical presentation, physical findings, results of pertinent lab/radiologic studies, differential diagnosis, and final diagnosis with discussion. [ABSTRACT FROM AUTHOR]

Background: Leonine facies (LF) is defined as displaying facial features similar to that of a lion with prominent convexities and furrowed creases. LF develops in a very small population of patients with cutaneous T-cell lymphoma.Objective: We aimed to study the clinicopathologic features and overall prognosis associated with LF in patients with mycosis fungoides and Sézary syndrome.Methods: We conducted a single-center retrospective study, reviewing 1338 patients with mycosis fungoides seen from 1987 to 2015 at a tertiary referral center for cutaneous T-cell lymphoma, and a systematic review of 14 patients in the literature.Results: We identified 10 patients with mycosis fungoides who developed LF. Folliculotropism was seen in all patients with LF who had facial biopsy specimens. Radiation was a beneficial therapy. Complete remission was achieved in 1 patient and overall 5-year survival was 26%. Systematic review of 10 additional patients showed that all patients with LF, including ours, had stage-IV disease and some degree of blood involvement, but not all met criteria for Sézary syndrome.Limitations: This was a retrospective study with a small sample size.Conclusion: LF is associated with stage-IV cutaneous T-cell lymphoma, is often accompanied by folliculotropism and blood involvement, and can be treated with local electron beam therapy. [ABSTRACT FROM AUTHOR]

Abstract: Aim: To evaluate the current treatment of mycosis fungoides (MF) and Sézary syndrome (SS) focusing on the role of radiotherapy (RT), its principles and indications, and the perspectives of the novel irradiation technologies. Background: MF and SS are rare lymphoproliferative diseases whose incidence is increasing. For a long time RT has been used as a single modality or in integrated treatment programs for these diseases. Materials and methods: The latest systematic reviews, primary studies and new diagnostic and treatment guidelines on MF and SS were analyzed. Clinical outcomes together with the technical aspects and the role of RT were also evaluated. Results: New data are available on pathogenesis, diagnostic criteria, classification and staging procedures for MF and SS and several local and systemic therapies are proposed. Localized RT can cure “minimal stage” MF while total skin electron beam irradiation (TSEI) may cure initial-stage disease and may offer important symptom relief (itch, erythroderma) in a more advanced setting. Despite its efficacy, RT is not largely used, mainly because of some technical difficulties but new RT technologies may be proposed to treat large skin surfaces. Conclusions: New treatment programs offer good results, with median survival of more than 12 years in early-stage MF, but the median survival of 2.5 years or less in advanced stages is still a challenge. RT remains an option for all stages with a good cost/effectiveness ratio in a curative or palliative setting. New RT technologies can overcome some technical problems of treating large skin surfaces. [Copyright &y& Elsevier]

Abstract: Aim: The aim of this paper was to present diagnostic methods helping in the recognition of mycosis fungoides (MF) and Sezary syndrome (SS). Background: Mycosis fungoides is the most common form of primary cutaneous T-cell lymphomas. It is characterized by a distinctive long-term course and malignant T-cell proliferation. MF diagnosis is not easy, mainly due to the atypical clinical presentation of the disease at an early stage. Materials and methods: Low specific changes, which can be observed at the histopathological examination. Initially, the skin lesions may resemble psoriasis, atopic dermatitis or chronic eczema. Patients are qualified according to the available, and generally accepted WHO-EORTC classification, based on a combination of clinical and histopathological markers. From a clinical point of view, it is also important to carry out the qualification according to the TNMB assessment, which allows to specify the stage of the disease, and is helpful in the monitoring of the course of disease and therapeutic effects. Results: In this paper we try to present currently available diagnostic methods. Conclusion: Diagnosis of MF and SS still causes many problems due to less characteristic changes in the early stage of disease and requires wide interdisciplinary knowledge. [Copyright &y& Elsevier]

Background: There is a paucity of effective therapies for patients with Sézary syndrome and advanced mycosis fungoides with peripheral blood involvement. Total skin electron beam (TSEB) radiation therapy is an extremely effective skin-directed therapy for these patients, but, until recently, it was thought not to signifcantly affect the peripheral blood malignant T-cell population. Objective: We conducted this study to determine if TSEB has therapeutic effect on the peripheral blood in patients with advanced mycosis fungoides and Sézary syndrome. Methods: All patients on stable medication regimens seen in our photopheresis facility who received TSEB therapy between January 2008 and October 2011 at Temple University Hospital, Philadelphia, PA, were analyzed retrospectively for improvement in the peripheral blood, as documented by flow cytometry. Results: Six of 11 patients achieved 50% or greater decrease in their peripheral blood malignant T-cell population after TSEB therapy, for an overall response rate of 55%. Within the group of patients who had a response in the skin, 67% also had a response in the peripheral blood. Limitations: This analysis is limited in 3 ways. First, the sample described is small. Second, the results may be confounded by the fact that each patient was on other systemic therapies in addition to TSEB, albeit stable pre-existing regimens. The time interval between completion of TSEB therapy and repetition of flow cytometry was not standardized among patients, which may result in an underestimation of the overall response to TSEB therapy. Conclusion: In patients with advanced mycosis fungoides and Sézary syndrome, the peripheral blood tumor burden may improve after treatment with TSEB. [ABSTRACT FROM AUTHOR]

Abstract: Dermatopathic lymphadenitis (DL) is a reactive lymphoid hyperplasia that commonly involves the regional lymph nodes in patients with chronic dermatoses, such as exfoliative dermatitis, toxic-shock syndrome, pemphigus, psoriasis, eczema, and, in severe cases, mycosis fungoides. Diagnosis of DL depends on the histopathologic characteristics of paracortical T-zone expansion with melanin, hemosiderin, or lipid-laden macrophages. Lymphadenopathies in patients with chronic dermatoses may be due not only to infections or malignancy, but also to reactive disorders such as DL. We describe the case of a 31-year-old woman with pemphigus vulgaris who has characteristic manifestations of DL. [Copyright &y& Elsevier]

Background: Sézary syndrome (SS), a leukemic variant of cutaneous T-cell lymphoma, is characterized by erythroderma and by atypical lymphocytes (Sézary cells) in peripheral blood. Although numerous studies have examined the range of disease in cutaneous T-cell lymphoma, a relative paucity of data exists to describe the long-term outcome of patients with SS. Objective: We sought to study long-term survival and prognostic factors of patients with SS. Methods: A retrospective chart review was conducted to identify patients with SS seen at Mayo Clinic from 1976 to 2010. Cox proportional hazards regression models, adjusted for age, were fit to evaluate factors associated with overall survival. Results: In total, 176 patients were identified with a clinicopathologic diagnosis of SS. Overall survival was 86.1% and 42.3% at 1 and 5 years, respectively, after diagnosis (median survival, 4.0 years). After adjustment for age, potential predictors of worse survival included lactate dehydrogenase level at presentation (hazard ratio [HR] 1.71; 95% confidence interval [CI] 1.18-2.47 per doubling), prior diagnosis of mycosis fungoides (HR 2.68; 95% CI 1.44-4.98), and the presence of T-cell receptor gene rearrangements in skin (HR 2.59; 95% CI 1.38-4.87) and in blood (HR 2.05; 95% CI 1.00-4.21). Limitations: This study is retrospective and represents a single academic center population. Conclusions: To our knowledge, this research evaluated the largest population of patients with SS studied to date. It shows that overall survival continues to be poor, with a median survival of 4.0 years after diagnosis. [ABSTRACT FROM AUTHOR]

Background: Spontaneous remission is recognized in mycosis fungoides (MF) and Sézary syndrome (SS). Objective: We analyzed the outcome of 44 patients with previously treated CD25-positive (CD25+), recurrent/persistent MF/SS randomly assigned to receive placebo as part of a phase III trial. Methods: This trial investigated the efficacy and safety of two doses of denileukin diftitox in patients with MF/SS who had received up to 3 prior therapies. The primary end point was overall response rate. Multivariate regression analyses were used to assess the relationship between baseline covariates and clinical outcomes. Results: The overall response rate was 15.9% for placebo recipients (complete response: 2.3%; partial response: 13.6%), reflecting the baseline rate of disease remission that can be expected in a clinical trial. The median progression-free survival (PFS) in the placebo arm was moderately short at 4.4 months compared with the active-agent arm but important to consider in the context of recent single-arm phase II studies of other therapies for MF/SS that report PFS of approximately 6 months. Multivariate analyses identified no significant effects of any baseline factors on either overall response rate or PFS, although there was a trend toward poorer PFS with advanced age. Because sepsis occurred significantly more often in the placebo arm versus the active-treatment arm, the role of antibiotics in causing remission cannot be discounted (6.8% vs 0%; P < .05). Limitations: This study had a relatively small sample size, yielding a wide 95% confidence interval. Conclusion: The results may serve as a useful comparator for other active-treatment studies of MF/SS that lack a placebo-control arm. [ABSTRACT FROM AUTHOR]

Background: Pruritus can be a distressing and even debilitating symptom for patients with cutaneous T-cell lymphoma (CTCL). To date, few studies have evaluated the pathophysiology of this symptom. Because of this, therapy for pruritus in CTCL has mainly relied on those therapies that target and treat the lymphoma. For patients living with CTCL that relapses or becomes refractory to treatment, and who continue to experience severe itch, this lymphoma-targeted treatment may not be enough to combat their pruritus. Therefore, other itch-targeted therapies are needed for use in this disease. Objective: We sought to evaluate the current evidence regarding the mechanism of action and treatments for pruritus associated with CTCL. Methods: An explicit and thorough search was restricted to all peer-reviewed literature available through MEDLINE (1950 to September 2011) and PubMed. Search terms used were “pruritus,” “cutaneous T-cell lymphoma,” “CTCL,” “mycosis fungoides,” “MF,” and “Sézary syndrome.” All studies that involved pruritus in CTCL, mycosis fungoides, or Sézary syndrome were evaluated by all 3 authors. Results: The current literature helps to identify therapies and possible mechanisms for treating patients with CTCL-associated pruritus. Limitation: Most studies were preclinical. Only studies involving mechanisms of action or treatment were included. Conclusion: A guideline is necessary to assist in the treatment of pruritus in CTCL and additional studies are necessary to uncover the exact mechanism or mechanisms of action. [ABSTRACT FROM AUTHOR]

Although Sézary syndrome (SS) represents an advanced stage of cutaneous T-cell lymphoma, this diagnosis presents a challenge even for the most experienced dermatologic clinicians. SS is characterized clinically by erythroderma, but can also be identified in the presence of specific histologic and peripheral blood findings. Erythrodermic cutaneous T-cell lymphoma can mimic a number of nonmalignant disorders with erythroderma, including pityriasis rubra pilaris, psoriasis, atopic dermatitis, and graft-versus-host disease. The diagnosis is made even more challenging because the histology of SS is often nonspecific and rarely pathognomonic. As a result, peripheral blood studies in patients with erythroderma are frequently informative in the diagnosis of SS. Peripheral blood abnormalities including elevated CD4/CD8 ratio, aberrant CD26, CD27 and CD7 expression, and T-cell clonality can all be used to help arrive at a diagnosis. This review evaluates current data on the usefulness and limitations of specific peripheral blood markers detected by flow cytometry and T-cell receptor gene rearrangement polymerase chain reaction. [Copyright &y& Elsevier]

In the management of patients with cutaneous T-cell lymphoma (CTCL), there are numerous distinct therapy options. Each of these therapies is discussed in terms of when to use it, what factors limit the success of the treatment, and what to expect. A menu is defined as a list of items from which to choose. The treatments for CTCL are presented in various menus where they are options for a particular goal in a particular setting of CTCL. The best recognized clinical scenarios of CTCL are those recognized by the staging system: limited patch plaque (T1), disseminated patch plaque (T2), erythroderma (T4), and tumor (T3). Each phase of the disease will have the menu of therapy options presented for a given goal of management. [Copyright &y& Elsevier]

Background: Lymphoma-associated hemophagocytic syndrome (LAHS) is a rare clinicopathological entity. It has been described with primary cutaneous lymphomas, mostly of the subcutaneous panniculitis-like T-cell type, and only once with cutaneous T-cell lymphoma (CTCL). Methods: We report the cases of 5 patients with epidermotropic CTCL who developed LAHS and died shortly thereafter. Unlike LAHS associated with systemic lymphomas, these CTCL-associated LAHS were late events, occurring several years after the initial lymphoma diagnosis. Limitations: The small number of patients reported renders definite conclusions difficult. Further reports would be needed to confirm our statements. Conclusion: LAHS is probably underdiagnosed in CTCL patients with acute inflammatory symptoms suggestive of infections but should be considered, especially when cytopenia and elevated triglyceride and ferritin levels are present. [ABSTRACT FROM AUTHOR]

Abstract: Exfoliative dermatitis or erythroderma in infancy is rare. Clinicians need to be alert to the possible diagnosis of Omenn syndrome (OS), a rare form of combined immunodeficiency in infants presenting with recurrent infections, erythroderma, lymphadenopathy, hepatosplenomegaly, eosinophilia, and increased serum IgE levels. OS is fatal unless treated by hematopoietic stem cell transplantation. We described a 26-day-old boy who had presented with diffusely thick scales on the scalp and some pustules on his face and trunk since birth. The rash was initially treated as presumed seborrheic dermatitis. At the age of 42 days, he developed extensive exfoliative dermatitis, lymphadenopathy, hepatosplenomegaly, and failure to thrive as well as eosinophilia and increased serum IgE levels. The histopathology of the skin and lymph node were consistent with the diagnosis of OS. He had been prepared for allograft bone marrow transplantation twice at 5 months and 7 months of age, but the procedure was not performed because of catheter-related sepsis. The patient eventually died of sepsis secondary to perianal cellulitis at 20 months of age. [Copyright &y& Elsevier]

Background: There is a need for standardized quantitative disease assessment measures in mycosis fungoides/Sézary syndrome. In 2005, a cutaneous T-cell lymphoma (CTCL)-severity index (SI) that not only measures disease extent (on a scale of 0-75) independent of the classic TNM(B) staging system but can also be used to estimate individual 5-year survival (SR5) was reported. Objective: We sought to assess the generalizability of the CTCL-SI/SR5 equation (SR5 equation) to predict prognosis in our cohort of patients with advanced mycosis fungoides/Sézary syndrome (n = 50, photopheresis service, 1984-2001). Methods: TNM(B) staging, CTCL-SI score (based on skin involvement, presence of tumors, lymph node/visceral/blood involvement), and SR5 (SR5 equation = 124 – 2 × [CTCL-SI]%) at initial diagnosis were calculated retrospectively and compared with overall survival by the Kaplan-Meier method. The prognostic significance of TNM(B) staging versus the CTCL-SI was determined by Cox proportional hazards models and Brier scores. Results: Patients had stage IIA to IVA disease with a median actuarial overall survival of 58 months. By disease stage, the overall 5-year survival was 70% (stage IIA), 48% (stage IIB-IIIB), and 36% (stage IVA). In our cohort, the CTCL-SI itself was predictive of overall survival (P = .028) but the SR5 equation was not predictive of survival (Brier score of 0.29). Limitations: Small sample size, single academic center population, and retrospective design are limitations. Conclusions: The CTCL-SI is a relatively simple-to-use quantitative tool that measures disease activity in all compartments (skin, nodes, blood, viscera) and has prognostic significance in multivariate analysis. The CTCL-SI may be a useful adjunct to the TNM(B) staging for tracking disease activity quantitatively in all disease compartments (skin, nodes, blood, viscera) in clinical practice and trials, but the predictive ability of the SR5 equation needs further validation at other centers in larger groups of patients. [Copyright &y& Elsevier]

Sézary 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. [ABSTRACT FROM AUTHOR]