Análisis de supervivencia en pacientes con micosis fungoide foliculotropa de un centro latinoamericano / Folliculotropic Mycosis Fungoides in a Latin American Hospital: Survival Analysis

Introducción La micosis fungoide foliculotropa es una variante de mal pronóstico y presentación clínica variada. Se ha planteado que la estadificación TNMB usada para esta neoplasia no es útil. En una propuesta reciente basada en aspectos clínicos e histológicos, se clasifica en enfermedad temprana y avanzada, encontrando diferencias pronósticas entre las 2categorías. El objetivo de este estudio fue comparar la supervivencia de estos 2 grupos en nuestra población. Materiales y métodos Se realizó un estudio observacional retrospectivo de serie de casos donde se evaluó la evolución clínica de los pacientes con micosis fungoide foliculotropa tratados en el Instituto Nacional de Cancerología entre el 2008 y el 2020, realizando un análisis comparativo de supervivencia entre aquellos que tienen enfermedad temprana y enfermedad avanzada. Resultados Se incluyó a un total de 21 pacientes, 11 de los cuales presentaban enfermedad temprana y 10 enfermedad avanzada. Se identificaron 7 decesos, todos ellos en los pacientes con enfermedad avanzada. La supervivencia global de la población total a 5 años fue del 62%, mientras que para la población con enfermedad avanzada fue del 40%. No hubo diferencias en la supervivencia según la estadificación TNMB. Conclusión La estadificación TNMB no es útil para los pacientes con una micosis fungoide foliculotropa. Por el contrario, la nueva clasificación clínico-patológica parece brindar información pronóstica fiable y permite tomar medidas terapéuticas acordes (AU)

Introduction Folliculotropic mycosis fungoides is a variant that has poor prognosis and a variable clinical presentation. Concerns have been expressed that the current TNMB staging of this tumor may not be useful. A recently developed classification system based on clinical and histologic variables classifies this tumor as early or advanced, a distinction found to correlate with prognosis. The aim of this study was to compare survival in FMF in Colombia between patients with early versus advanced tumors. Material and methods Retrospective, observational study of clinical course and outcomes in patients with FMF treated at the National Cancer Institute of Colombia between 2008 and 2020. Survival was compared between early and advanced disease. Results Twenty-one patients (11 with early FMF and 10 with advanced FMF) were studied. Seven patients, all with advanced disease, died. Survival at 5 years was 62% overall and 40% for patients with advanced FMF. No differences were observed when survival was analyzed according to TNMB stage. Conclusions TNMB staging is not useful in FMF. The new classification system based on clinicopathologic features appears to provide reliable information for assessing prognosis and guiding treatment decisions (AU)

[Translated article] Folliculotropic Mycosis Fungoides in a Latin American Hospital: Survival Analysis / Análisis de supervivencia en pacientes con micosis fungoide foliculotropa de un centro latinoamericano

Introduction Folliculotropic mycosis fungoides is a variant that has poor prognosis and a variable clinical presentation. Concerns have been expressed that the current TNMB staging of this tumor may not be useful. A recently developed classification system based on clinical and histologic variables classifies this tumor as early or advanced, a distinction found to correlate with prognosis. The aim of this study was to compare survival in FMF in Colombia between patients with early versus advanced tumors. Material and methods Retrospective, observational study of clinical course and outcomes in patients with FMF treated at the National Cancer Institute of Colombia between 2008 and 2020. Survival was compared between early and advanced disease. Results Twenty-one patients (11 with early FMF and 10 with advanced FMF) were studied. Seven patients, all with advanced disease, died. Survival at 5 years was 62% overall and 40% for patients with advanced FMF. No differences were observed when survival was analyzed according to TNMB stage. Conclusions TNMB staging is not useful in FMF. The new classification system based on clinicopathologic features appears to provide reliable information for assessing prognosis and guiding treatment decisions (AU)

Introducción La micosis fungoide foliculotropa es una variante de mal pronóstico y presentación clínica variada. Se ha planteado que la estadificación TNMB usada para esta neoplasia no es útil. En una propuesta reciente basada en aspectos clínicos e histológicos, se clasifica en enfermedad temprana y avanzada, encontrando diferencias pronósticas entre las 2categorías. El objetivo de este estudio fue comparar la supervivencia de estos 2 grupos en nuestra población. Materiales y métodos Se realizó un estudio observacional retrospectivo de serie de casos donde se evaluó la evolución clínica de los pacientes con micosis fungoide foliculotropa tratados en el Instituto Nacional de Cancerología entre el 2008 y el 2020, realizando un análisis comparativo de supervivencia entre aquellos que tienen enfermedad temprana y enfermedad avanzada. Resultados Se incluyó a un total de 21 pacientes, 11 de los cuales presentaban enfermedad temprana y 10 enfermedad avanzada. Se identificaron 7 decesos, todos ellos en los pacientes con enfermedad avanzada. La supervivencia global de la población total a 5 años fue del 62%, mientras que para la población con enfermedad avanzada fue del 40%. No hubo diferencias en la supervivencia según la estadificación TNMB. Conclusión La estadificación TNMB no es útil para los pacientes con una micosis fungoide foliculotropa. Por el contrario, la nueva clasificación clínico-patológica parece brindar información pronóstica fiable y permite tomar medidas terapéuticas acordes (AU)

Association of marital status with stage and survival in patients with mycosis fungoides: A population-based study.

BACKGROUND: Previous studies have shown that marital status was associated with stages and survival in patients with melanoma or Merkel cell carcinoma. To date, the impacts of marital status on stage and survival in patients with mycosis fungoides (MF) have not been determined yet. METHODS: A total of 3375 eligible cases diagnosed from 2004 to 2015 were included from the Surveillance, Epidemiology, and End Results (SEER) database. Association of marital status with stage and survival in patients with MF was analyzed. RESULTS: Married patients were more likely to be diagnosed at T1 stage (p = 0.041). And married patients were less likely to present with lymph node involvement (p = 0.007). More favorable overall survival (p < 0.001) and cancer-specific survival (p < 0.001) were demonstrated in married patients as compared with divorced patients or widowed patients. A clinically feasible prognostic model including marital status, age, sex, race, and stage at presentation was constructed. CONCLUSION: Married marital status was associated with earlier stage at diagnosis and longer survival compared with divorced or widowed marital status in patients with MF.

Mycosis Fungoides and Sézary Syndrome: Updates and Review of Current Therapy.

OPINION STATEMENT: While most patients with early-stage mycosis fungoides (MF) follow an indolent course, patients with advanced-stage MF/Sézary syndrome (SS) have a poor prognosis with a median survival of less than 5 years. Although there are a number of treatments currently available, achieving and maintaining a durable response remain challenging, especially in advanced-stage MF/SS. The choice of frontline therapy is dependent on the stage of disease. For early-stage MF, the treatment concept is to control skin lesions mainly by skin-directed therapies, such as topical therapies, phototherapies, and radiotherapies. For advanced-stage MF/SS, systemic treatments by biological or targeted therapies including bexarotene and interferon either alone or in combination are tried first, with more immunosuppressive chemotherapies being reserved for refractory or rapidly progressive disease. Recent improvements in biological or targeted therapies include brentuximab vedotin and mogamulizumab. When biopsy samples have 10% or more CD30-positive malignant cells, brentuximab vedotin, an anti-CD30 antibody conjugated to monomethyl auristin E, can be a desirable treatment option. For cases with blood involvement, mogamulizumab, an antibody binding to C-C chemokine receptor 4, is effective with high response rates. In the refractory setting, alemtuzumab, histone deacetylase inhibitors, pralatrexate, gemcitabine, and doxorubicin are considered as the treatment option. Because only allogeneic hematopoietic stem cell transplantation can offer a chance of cure with durable complete remission, advanced-stage patients with a markedly short life expectancy should be evaluated for eligibility. Given that there are few randomized controlled studies in the literature, it is necessary to investigate which therapy is preferable for each patient with MF/SS by comparative prospective trials.

Role of chromatin assembly factor-1/p60 and poly [ADP-ribose] polymerase 1 in mycosis fungoides.

Mycosis fungoides (MF) represents the most common type of cutaneous lymphoma. In the majority of patients, the disease has a slow evolution and a protracted course; however, a subset of patients shows poor oncologic outcomes. Unfortunately, there are no reliable prognostic markers for MF, and the currently available treatments are only effective in a minority of patients. This study aimed to evaluate the expression and clinical significance of PARP-1 and CAF-1/p60 in MF. Sixty-four MF representatives of the different stages of disease were assessed by immunohistochemistry for PARP-1 and CAF-1/p60. The association of PARP-1 and CAF-1/p60 with the MF stage and outcome was assessed by using Fisher's exact test and Kaplan-Meier survival analysis with the Log-rank test; a p value < 0.05 was considered significant. PARP-1 was overexpressed in 57.9% of MF and was significantly associated with a MF stage > II (p = 0.034) but not with the risk of death (p = 0.237). CAF-1/p60 was overexpressed in 26.8% of MF and was significantly associated with decreased overall survival (p < 0.001) but not with the MF stage (p = 1). A significant association was found between PARP-1 overexpression and CAF-1/p60 overexpression (p = 0.0025). Simultaneous overexpression of PARP-1 and CAF-1/p60 was significantly associated with decreased overall survival (p < 0.001), although less strongly than CAF-1/p60 alone (χ2 = 14.916 vs 21.729, respectively). In MF, PARP-1 is overexpressed in advanced stages, while CAF-1/p60 is overexpressed in the cases with shorter overall survival, appearing as a significant prognostic marker. A role for PARP-1 inhibitors and anti-CAF-1/p60 targeted therapy may be reasonably hypothesized in MF.

Differential SATB1 Expression Reveals Heterogeneity of Cutaneous T-Cell Lymphoma.

SATB1 is an important T-cell specific chromatin organizer in cutaneous T-cell lymphoma, whereas its expression and function in mycosis fungoides (MF) remain ambiguous. Our study aimed to investigate the clinicopathological significance of SATB1 in a cohort of 170 patients with MF. SATB1 expression was heterogeneous among the patients with MF in each clinical stage. High SATB1 expression was associated with epidermal hyperplasia, eosinophil infiltration, less large-cell transformation, and favorable prognosis in MF cases. SATB1 and CD30 coexpression distinguished cutaneous CD30+ lymphoproliferative disorders from MF large-cell transformation. SATB1 silencing in MF lines showed that SATB1 upregulated the genes involved in eosinophil recruitment, including signal transducer and activator of transcription 3 and IL13, and downregulated the genes in cell-cycle progression, which may explain the inferior prognosis for low SATB1-expressing cases. Moreover, SATB1 was inversely correlated with PD-1 expression, indicating an exhausted status of SATB1-negative malignant T cells. SATB1 was positively correlated with toll-like receptors expression, suggesting innate immune activation in high SATB1-expressing MF cases. Therefore, variable SATB1 expression promotes heterogeneity in pathology and clinical outcome of patients with MF.

Mycosis fungoides and Sézary syndrome: Australian clinical practice statement.

Primary cutaneous lymphomas represent a heterogeneous group of T- and B-cell lymphomas with distinct clinical presentations, histopathologic features, treatment approaches and outcomes. The cutaneous T-cell lymphomas, which include mycosis fungoides and Sézary syndrome, account for the majority of the cutaneous lymphomas. This Clinical Practice Statement is reflective of the current clinical practice in Australia. An expanded form of the Clinical Practice Statement (and updates), along with helpful patient resources and access to support groups, can be found at the following (http://www.australasianlymphomaalliance.org.au).

Phenotypical Markers, Molecular Mutations, and Immune Microenvironment as Targets for New Treatments in Patients with Mycosis Fungoides and/or Sézary Syndrome.

Primary cutaneous lymphomas encompass a wide spectrum of rare lymphoproliferative disorders originating in the skin, among which, mycosis fungoides (MF) is the most common subtype. The treatment of this disease is based on skin-directed therapies eventually in association with biologic response modifiers in the early phases, whereas in patients with the advanced stages, several therapeutic strategies can be used including mono and/or polychemotherapy and bone marrow transplantation. In recent years, the identification of specific markers (phenotypical, immunological, and molecular) has led to the development of several studies (including two randomized phase III trials). The results of these studies are modifying our therapeutic strategy toward a personalized treatment approach in which the clinical characteristics of the patients and tumor-node-metastasis-blood stage are considered together with the expression of specific markers (i.e., a CD30-positive expression for the use of brentuximab vedotin). This review will provide a comprehensive scenario of the main phenotypical, molecular, and immunological markers related to MF pathogenesis and disease evolution, which could represent the target for the development of innovative effective treatments in this disease.

The immunopathogenesis and immunotherapy of cutaneous T cell lymphoma: Pathways and targets for immune restoration and tumor eradication.

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.

Survival prediction model for patients with mycosis fungoides/Sezary syndrome.

Aim: A nomogram was constructed to forecast the overall survival (OS) of patients with mycosis fungoides/Sezary syndrome. Patients & methods: The clinicopathological information of patients was obtained from the Surveillance, Epidemiology and End Results (SEER) database. A model was established based on the independent prognostic factors. Predictive ability of the model was evaluated with the concordance index and calibration curves. Risk stratification was conducted for patients with similar tumor node metastasis (TNM) stages. Results: The model included 1997 eligible patients and seven prognostic factors for OS. The concordance index of the nomogram was 0.84 in the training and external validation cohorts, which indicated good predictive ability of the model and reliability of the results. The high agreement between the model predictions and actual observations was identified by calibration curves, which demonstrated the prediction accuracy of the model. Risk stratification displayed significant differences for patients with similar TNM stages, which suggested that the OS of patients with similar TNM stages could be further distinguished. Conclusion: We established a reliable nomogram to predict the OS of patients with mycosis fungoides/Sezary syndrome, which highlighted the advantages of nomograms over the conventional TNM staging system and promoted the application of individualized therapeutic strategies.

Outcome of allogeneic hematopoietic stem cell transplantation for mycosis fungoides and Sézary syndrome.

Although allogeneic hematopoietic stem cell transplantation (HSCT) has been reported to provide prolonged remission of relapsed/refractory mycosis fungoides (MF) and Sézary syndrome (SS), its role has not been fully evaluated. Here, the outcomes of allogeneic HSCT for patients with MF/SS were retrospectively evaluated by using the registry database of the Japan Society for Hematopoietic Cell Transplantation. Forty-eight patients were evaluable and enrolled in the analysis. Median age was 45.5 years. Eighteen patients (38%) received myeloablative conditioning, and 33 (69%) received HSCT from an alternative donor. Disease status was complete or partial response in 25% of the patients and relapsed or refractory in the others. At the time of analysis, 18 patients were alive, with a median follow-up of 31.0 months (range, 3.8-31.1). Three-year overall survival (OS) and progression-free survival (PFS) were 30% (95%CI, 16-45%) and 19% (95%CI, 9-31%), respectively. Disease progression was not observed later than 17 months after transplantation. Both disease status and performance status at transplant significantly affected OS and PFS. Although our findings suggest that allogeneic HSCT provides long-term PFS in patients with MF/SS, the timing of transplantation should be decided carefully based on the disease status and the patient's condition in order to improve the outcome.

Efficacy of Allogeneic Hematopoietic Cell Transplantation in Cutaneous T Cell Lymphoma: Results of a Systematic Review and Meta-Analysis.

Mycosis fungoides and Sézary syndrome are the most common types of primary cutaneous T cell lymphomas. The clinical presentation of mycosis fungoides is generally indolent, whereas Sézary syndrome represents a more aggressive disease variant. Stage at diagnosis is the most important determinant of long-term survival outcome. Although most patients present with early-stage disease, those who develop progressive disease or have an advanced stage represent a therapeutic challenge because of a lack of effective therapies. Allogeneic hematopoietic cell transplantation (allo-HCT) has been used as a potentially curative treatment modality with encouraging long-term outcomes. However, a lack of randomized controlled data remains, and the published literature is limited to mostly retrospective studies. We performed a comprehensive search of the medical literature using PubMed/Medline, EMBASE, and Cochrane reviews on September 13, 2018. We extracted data on clinical outcomes related to benefits (overall [OS] and progression-free [PFS] survival) and harms (relapse and nonrelapse mortality [NRM]) independently by 2 authors. Our search strategy identified 289 references. Five studies (266 patients) were included in this systematic review and meta-analysis. Reduced-intensity and nonmyeloablative regimens were more commonly prescribed (76%). Mobilized peripheral blood stem cells were the preferred graft source (78%). The pooled OS and PFS rates were 59% (95% confidence interval [CI], 50% to 69%) and 36% (95% CI, 27% to 45%), respectively. Pooled relapse rate was 47% (95% CI, 41% to 53%) and pooled NRM rate 19% (95% CI, 13% to 27%). Results of this systematic review and meta-analysis show that allo-HCT yields encouraging OS and PFS rates; however; relapse remains a significant cause of allo-HCT failure. Novel strategies to further improve outcomes should focus on offering allo-HCT before the development of resistant disease and reducing relapse by incorporating post-transplant maintenance therapies.

Outcomes and prognostic factors in African American and black patients with mycosis fungoides/Sézary syndrome: Retrospective analysis of 157 patients from a referral cancer center.

BACKGROUND: The prevalence of mycosis fungoides/Sézary syndrome (MF/SS) is higher in the black population than in the white population in the United States and worse outcomes have been observed in black patients. OBJECTIVE: To describe the outcomes and to identify prognostic factors in African American and black patients with MF/SS. METHODS: Clinical features and follow-up data were analyzed in 157 self-identified African American or black patients seen during 1994-2018. RESULTS: We included 122 patients with early stage MF and 35 patients with advanced-stage disease (median follow-up of 25 months). Overall, >80% of the patients who died from disease or progressed had erythema or hyperpigmentation without hypopigmentation. Patients with hypopigmentation, either as the sole manifestation or in combination with other lesions, had better overall survival (P = .002) and progression-free survival (P = .014). Clinical stage, TNMB classification, plaque disease, and elevated serum lactate dehydrogenase were also significantly associated with outcomes. Demographic and socioeconomic parameters were not associated with prognosis. LIMITATIONS: A retrospective study at a single cancer center. CONCLUSION: MF/SS manifestations and outcomes in African American and black patients are heterogeneous. Demographic and socioeconomic factors do not seem to have a prognostic role, while clinical characteristics might help in the stratification of risk of progression and shorter survival, allowing for individually tailored therapeutic interventions.

Early-stage Mycosis Fungoides: Epidemiology and Prognosis.

Most patients with mycosis fungoides are diagnosed with early-stage disease. However, prevalence of early-stage disease is unknown, and evidence of its burden is scarce. The aim of this study is to estimate the prevalence of early-stage mycosis fungoides, how long patients live with early-stage disease and to characterise these patients. Data were obtained from 4 key publications and from US cancer registries (Surveillance, Epidemiology and End Results Program; SEER). The derived incidence of early-stage mycosis fungoides was 0.26/100,000 (UK), 0.29/100,000 (US) and 0.38/100,000 (US-SEER) and the prevalence was 4.8/100,000 (UK), 5.2/100,000 (US) and 6.6/100,000 (US-SEER). Early-stage disease may last for 18 years. From SEER registries, 3,132 were diagnosed at early stage (mostly stage IA). Median age at diagnosis was 58 years. Compared with stage IA, the relative risk of death was 1.3 for stage IB and 3.5 for stage IIA. We confirm the rarity of early-stage mycosis fungoides, a differential prognosis and the potential for elevated burden of disease.

Radiotherapy Eradicates Malignant T Cells and Is Associated with Improved Survival in Early-Stage Mycosis Fungoides.

PURPOSE: Mycosis fungoides is the most common subtype of cutaneous T-cell lymphoma. Skin-directed treatments often improve but do not cure mycosis fungoides skin lesions. The purpose of this study was to (i) assess whether remission was associated with malignant T-cell clone depletion at treated sites using either low-dose radiotherapy (LDRT, 8 Gy) or topical steroids and (ii) assess whether a clone-ablative therapy, like LDRT, is associated with overall survival in patients with high-risk early-stage CTCL. EXPERIMENTAL DESIGN: Pre- and posttreatment biopsies from 20 lesional skin samples of 18 patients with mycosis fungoides who received either 8 Gy LDRT (n = 16) or topical steroids (n = 4) underwent high-throughput T-cell receptor sequencing of the TCRB gene to quantify the malignant T-cell clone. For the retrospective chart review, overall survival of 47 high-risk early-stage patients was compared between patients who did or did not receive radiation. RESULTS: LDRT eradicated the clone in 5 of 16 lesions and reduced it >90% in 11 of 16; there were no recurrences in these lesions. Patients treated with topical steroids appeared to clinically improve, but the malignant clone persisted. We found that the number of residual malignant T cells predicted lesion recurrence. A retrospective review showed that early-stage high-risk patients who received radiation as part of their treatment regimen had prolonged overall survival compared with patients who did not. CONCLUSIONS: These findings demonstrate that LDRT can eradicate malignant T cells in mycosis fungoides, provides robust disease control, and is associated with improved survival in high-risk early-stage patients.

Plaque stage folliculotropic mycosis fungoides: histopathologic features and prognostic factors in a series of 40 patients.

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a distinct variant of mycosis fungoides. Recent studies recognized indolent and aggressive subgroups of FMF, but there is controversy how patients presenting with plaques should be classified. The present study describes the histopathologic features of 40 FMF plaques. The aim of the study was to identify risk factors for disease progression and poor outcome in this group. METHODS: Clinical, histopathological, and immunophenotypical data from 40 patients with plaque stage FMF were reviewed and analysed for risk factors for disease progression and survival. RESULTS: After a median follow-up of 80 months, disease progression occurred in 20 of 40 patients. Percentage of atypical cells, cell size, percentage of Ki-67+ cells, and co-existent interfollicular epidermotropism, but not the extent of perifollicular infiltrates, were associated with disease progression and reduced survival, while extensive follicular mucinosis was associated with increased survival. CONCLUSIONS: This study underlines that FMF patients presenting with plaques represent a heterogeneous group and that a subgroup of these patients may have an indolent clinical course. It further shows that histological examination is a valuable tool to differentiate between indolent and aggressive disease.

Racial Disparities in the Clinical Presentation and Prognosis of Patients with Mycosis Fungoides.

OBJECTIVE: Racial and gender disparities in mycosis fungoides (MF) are understudied. The objective of this study was to test the hypothesis that worse prognosis in blacks with MF is mediated by higher disease stage at diagnosis and by earlier disease onset in black females. METHODS: We conducted retrospective chart review of 337 patients with clinically-suspected MF seen at Johns Hopkins between 2003 and 2018, requiring biopsy-proven disease for study inclusion. Patient demographics, initial stage/percent body surface area (BSA) involvement, pathology type, flow cytometry results, and treatment regimens were recorded. RESULTS: Of 303 patients with confirmed MF, 166 (55%) were white, 107 (35%) black, 10 (3.3%) Middle Eastern, 6 (2.0%) Asian, and 14 (4.6%) Hispanic/other. Blacks were 3 times as likely (95% CI: 1.2, 8.0) to have Stage 2 disease to have Stage 2 disease at diagnosis as compared to whites as whites. In females, blacks were younger at diagnosis (p = 0.003) and at death (p = 0.008) compared to whites. In males, blacks had 4 times the odds of late-stage disease (p = 0.017) and presented with 19% greater BSA involvement on average compared to whites (p < 0.001). CONCLUSIONS: Compared to their white counterparts in this cohort, black males were diagnosed with MF at a higher stage with greater skin involvement while black females were diagnosed and died earlier. Earlier recognition of MF in skin of color and closer follow-up of black females with early-onset, aggressive disease may help to mitigate disparities in outcomes.

Clinical and Histological Characteristics of Mycosis Fungoides and Sézary Syndrome: A Retrospective, Single-centre Study of 43 Patients from Eastern Denmark.

Diagnosis of mycosis fungoides and Sézary syndrome can be very challenging. Clinical and histopathological data for patients with mycosis fungoides and Sézary syndrome in Denmark are limited. A retrospective study was performed in Region Zealand, Denmark from 1990 to 2016. A total of 43 patients with mycosis fungoides or Sézary syndrome were identified during the period. At the time of diagnosis the patients' mean age was 64.3 years and 74.5% had early-stage (≤IIA) disease. The mean time from onset of skin disease to diagnosis was 4.4 years. Surprisingly, 43% progressed to a higher disease stage, and risk of disease progression was higher for stage IB than IA (p = 0.01). All cases displayed some degree of epidermotropism and the infiltrates consisted of pleomorphic lymphocytes with a T-helper (CD4+/CD8-) phenotype. This study describes, for the first time, all aspects of clinical and histopathological findings in patients with mycosis fungoides and Sézary syndrome in a well-characterized Danish cohort.

Mycosis Fungoides: A 10-year Turkish Experience.

Mycosis fungoides is the most common primary cutaneous T cell lymphoma, characterized by erythematous patches and plaque lesions with slow progression to cutaneous tumors or extracutaneous involvements in some patients. We aimed to evaluate the clinical characteristics, treatment responses, disease courses, and mortality rates of our MF cases. The data of 100 patients with MF were retrospectively examined from medical records in our clinic between January 2005 and January 2015. Demographic and clinical characteristics of the patients, disease stage, treatment protocols, response to treatment, recurrence, progression, and mortality rates were recorded. The male to female ratio in patients was 1.2. Mean age at onset of disease was 46, and duration of disease ranged from one to 42 years. At time of diagnosis 31 patients were at stage 1A, 31 at stage 1B, 30 at stage 2A, 2 at stage 2B, 1 at stage 3, and 5 at stage 4. Stable disease was observed in 35% of patients, progression in 10%, relapse in 27%, and complete response in 28%. Large cell transformation was found in 3 patients and additional malignity in 11. Thirty-seven patients (37%) were still surviving disease-free. 10 patients had died, three of them due to disease-related conditions. The most common first-line therapy in our study was phototherapy. It was applied to 87% of patients from stage 1A. Our results are generally consistent with current literature, but disease progression and disease-specific mortality rates were significantly lower than the literature, probably due to early phototherapy.