Cases Of Mycosis Fungoides Research Articles

Narrowing of the Racial Disparities Gap in Survival of Patients with Mycosis Fungoides: A Longitudinal Analysis of the SEER Database (1988 to 2011)

Introduction: Mycosis Fungoides (MF) is the most common subtype of cutaneous T-cell lymphomas. Prior Studies have identified Black race as a risk factor for earlier age at diagnosis, more advanced stages at time of diagnosis and poor prognosis in patients with MF. Data examining differences in racial disparities outcomes over time are limited.Objective: This retrospective analysis aims to examine if the racial disparities in survival outcomes of MF patients have improved over time.Subjects and Methods: Using the United States Surveillance, Epidemiology and End Results (SEER) 1988-2011 public use database, we examined survival patterns for patients with MF (with the code of 9700) between 1988 and 2011. Cases were divided into three cohorts based on the year of diagnosis; “1988 - 1995”, “1996 - 2003”, and “2004 - 2011”. Univariable and multivariable analysis were conducted to assess for factors significantly associated with the overall survival. The nonparametric estimates of the survival distribution function, Kaplan and Meier survival curves, and Cox proportional hazards model were used to investigate the factors affecting the survival time.Results: From 1988 to 2011, a total of 2896 cases of MF were identified with a median follow-up of 60 months. The difference in the survival time between the years of diagnosis 1988-1995 and 2004-2011 is significant (p-value=0.05). The parameter estimate of the Cox proportional hazards model for the “1988-1995” and the “2004-2011” period as a reference is also significant (p-value = 0.024) and the hazard ratio (HR) is 1.407, which means that patients diagnosed in 1988-1995 were 1.4 times likely to die from the disease compared to the patients diagnosed in 2004-2011 (i.e. patients in 1988-1995 were more likely to not survive than in 2004-2011) (Table 1 and 2). There is no significant difference in the survival of the patients between “1996-2003” and “2004-2011” (p-value 0.998), Cox model estimate is not significant (p-value = 0.178), and the HR is 0.94 (Table 1 and 2). For the time period 1988-1995, the survival of Black patients was inferior to White (p= 0.0339), Asians (p=0.001), and other races (p=0.0011); Figure 2 and Table 3. For the time period 1996-2003, there was no difference in survival across races (p-value=0.7599); Figure 3 and Table 3. For the time period of 2004-2011, survival of Black patients was similar to White (p-value=1) but again inferior to Asian (p-value=0.05) and other races (p-value=0.09); Figure 4 and Table 3. Across the entire time period of 1998-2011, the survival of Black patients was inferior to White (Chi-square=6.59 and p-value=0.0084); Figure 5. The survival gap between Black and White patients seems to be obliterated in subsequent; “1996 - 2003” and “2004 - 2011” vs 1988-1995 (Figures 3 and 4) due to improvements in survival of Black patients over time (Figure 6) while the survival of White patients remained rather steady over time (Figure 7).Conclusions: Our study demonstrated that Black race was significantly correlated with poorer survival in patients with MF. The etiology of this poorer prognosis can be related to access to medical care, socioeconomic disparities, or possibly difference in disease biology and immune response. Despite the persistent pattern of lower survival across all time periods, the gap in survival between White and Black races seems to be narrowing overtime. [Display omitted] DisclosuresWilliam: Dova Pharmaceuticals: Research Funding; Incyte: Research Funding; Kyowa Kirin: Consultancy; Merck: Research Funding; Guidepoint Global: Consultancy.

Targeted Deep Sequencing of Mycosis Fungoides Reveals Intracellular Signaling Pathways Associated with Aggressiveness and Large Cell Transformation.

Simple SummaryIn order to decipher the molecular mechanisms of large cell transformation (LCT) in mycosis fungoides (MF), we screened 51 longitudinally obtained skin samples of mycosis fungoides patients (n = 27) with versus without large-cell transformation by the means of targeted deep sequencing in close clinicopathological correlation. The analysis of longitudinally obtained tissue revealed a dynamic mutational profile in the context of an evolutionary selection processes with the example of PLCG1 alterations. In patients with an aggressive clinical course, we detected high mutational heterogeneity revealing the highest frequency of mutations in patients with LCT. The affected genes included members of the JAK/STAT signaling pathway and epigenetic modifiers. The key findings of our analysis included recurrent activating RAS mutations (KRAS and NRAS) being exclusively present in LCT MF; what is of note is that these molecular aberrations were already present in early stages; thus, RAS mutations in MF exhibit a prognostic marker for a higher risk of relapse and progression and restricted prognosis. As RAS mutated tumors are currently in the focus of novel targeted treatment options in several clinical trials, such personalized treatment modalities might offer novel therapeutic options for RAS mutated MF patients.Introduction: Large-cell transformation (LCT) of mycosis fungoides (MF) has been associated with a higher risk of relapse and progression and, consequently, restricted prognosis. Its molecular pathogenesis has not been elucidated yet. Materials and Methods: In order to address molecular mechanisms of LCT, we performed hybrid capture panel-based sequencing of skin biopsies from 10 patients suffering from MF with LCT versus 17 patients without LCT including follow-up biopsies during clinical course, respectively (51 samples in total). The analyzed patients were attributed to three different groups based on the presence of LCT and clinical behavior. Results: While indolent MF cases without LCT did not show pathogenic driver mutations, a high rate of oncogenic alterations was detected in patients with LCT and aggressive clinical courses. Various genes of different oncogenic signaling pathways, including the MAPK and JAK-STAT signaling pathways, as well as epigenetic modifiers were affected. A high inter-individual and distinctive intra-individual mutation diversity was observed. Oncogenic RAS mutations were exclusively detected in patients with LCT. Conclusion: Our data demonstrate that LCT transition of MF is associated with increased frequency of somatic mutations in cancer-associated genes. In particular, the activation of RAS signaling—together with epigenetic dysregulation—may crucially contribute to the molecular pathogenesis of the LCT phenotype, thus conveying its adverse clinical behavior.

Tre-P-18 - The use of small doses of gemcitabine in the treatment of drug-resistant forms of mycosis fungoides/Sézary syndrome

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most frequent cutaneous T-cell lymphomas. These tumors have a chronic progradient course. Hitherto there is no evidence of effective therapy, particularly of the late-stage of the diseases. About 30% of the patients are not effected by interferon, radiotherapy and combined treatment regimens. Patients' age (median age 62 years) limits therapy options. Several lines of previous treatment could lead to tumor resistance and higher accumulated toxicity. Thus, use of potentially effective chemotherapeutic agents in lower doses will reduce myelotoxicity and expand the cohort of patients. Gemcitabine is an effective medication in treatment of MF/SS. Its efficacy is preserved in small doses with prolonged duration of infusion and allows to decrease myelotoxicity. It was firstly discovered in therapy of solid tumors. In current study 14 patients were involved with resistant forms of MF/SS (10 patients with MF, 3 with SS, and 1 case of MF with transformation into large cell lymphoma). We used 6-hour infusion of gemcitabine at dosage of 250 mg/m². 86% of patients achieved a general response: 29% complete remission, 50% partial remission, 21% stabilization of disease, 14% (2 patients) progression. Present study showed promising results in treatment of patients with advanced stages of MF/SS which had at least two preceding lines of therapy without an effect. Common response (PR/CR) was received in high percent of cases. This treatment protocol is reproducible in elderly patients. For long-term conclusions further studies are required.

Hist-P-01 - Evaluation of the PCR-based T cell receptor-β clonality test in the diagnosis of early mycosis fungoides

<h3>Background:</h3> T-cell receptor (TCR) clonality is important for mycosis fungoides (MF) diagnosis. Routine clonality analysis is performed using a polymerase chain reaction (PCR) TCR-γ assay; yet with this method 10–50% of T-cell lymphomas escape detection. TCR-β gene rearrangement is an additional assay. Data about its efficacy is controversial. <h3>Objective:</h3> Evaluate the role of TCR-β assay in the diagnosis of early MF. <h3>Methods:</h3> A retrospective study of 61 skin biopsies, 20 from MF patients, 30 from patients suspected to have early MF, and 11 from patients with chronic inflammatory skin disease. <h3>Results:</h3> Monoclonality was detected in 16/20 (80%) MF cases; 15 (75%) with TCR-β and 12 (60%) with TCR-γ assay. Of the 30 suspected early MF cases, 14 demonstrated monoclonality, all of which with TCR-β and 5 with TCR-γ assay. None of the chronic inflammatory condition samples showed monoclonality. Therefore, TCR-β clonality assay was more sensitive in early MF than TCR-γ (83% vs. 43%, P=0.002). <h3>Limitations:</h3> A retrospective, relatively small study. <h3>Conclusion:</h3> TCR-β demonstrated a higher sensitivity rate compared with TCR-γ in early-stage MF. The combined use of the TCR-β and TCR-γ clonality tests can significantly improve the diagnosis rate of early-stage MF.

Epi-O-01 - Incidence of mycosis fungoides and Sézary syndrome in the Netherlands in the last two decades

Cutaneous T-cell lymphomas (CTCLs) are a heterogeneous group of non-Hodgkin lymphomas that differ greatly in clinical presentation and prognosis. Mycosis fungoides (MF) is the most frequent subtype. Sézary syndrome (SS) is less prevalent but has a much more unfavourable prognosis. Previous studies have shown that the incidence of CTCL has tripled between 1970 and 2000 but since then has stabilized. However, data on the incidence of MF and SS in the Netherlands have never been published. The aim of the present study was to estimate the changes in incidence of newly diagnosed MF and SS in the Netherlands over the last 20 years. Annual incidence rates were retrieved from the Dutch Cutaneous Lymphoma Registry (DCLR) between January 2000 and December 2019. In all cases, the diagnosis was based on the clinicopathological criteria of the World Health Organization European Organisation for Research and Treatment of Cancer classification and confirmed by an expert panel of dermatologists and pathologists at a periodical meeting of the Dutch Cutaneous Lymphoma Working Group. We calculated the number of registered patients with MF and SS per 100 000 persons, corrected for the size of the Dutch population. 1044 patients with MF, including 238 patients with folliculotropic MF (FMF), and 93 patients with SS were included. In 2000, the corrected number of cases of MF was 0.19 per 100 000 persons, while this was 0.35 per 100 000 in 2010 and 0.46 per 100 000 persons in 2019. This means that a 2.42-fold increase, corrected for Dutch population growth, was seen between 2000 and 2019 for the incidence of MF in the Netherlands. In 2000, 2010 and 2019 the corrected incidences for SS were 0.013, 0.018 and 0.075 per 100 000 persons, respectively. The overall increase for SS was 6.0-fold corrected for the Dutch population between 2000 and 2019. In summary, a significant increase of patients with classical MF, FMF and SS included in the DCLR was seen over the past 20 years. In contrast to previous studies that suggest a stabilization since 2000, this study shows that the incidence of patients with MF and SS in the Netherlands increased 2.42-fold over the past two decades.

Assessment of The Diagnostic Value of TOX Versus CD3 Immunohistochemical Markers in Detection of Early Mycosis Fungoides Cases

Background: Primary cutaneous lymphoma (PCL) with clonal proliferation of atypical CD4+ skin-homing T lymphocyte cells is called Mycosis Fungoides (MF). TOX staining is observed in subtypes of PCL, as MF & Sezary Syndrome (SS) in comparison to controls. Early MF is difficult to diagnose, & its distinction from inflammatory diseases may be impossible. Objective: This study aimed to evaluate the expression of the TOX versus CD3 as a diagnostic marker for early MF. Patients and methods: retrospective-cross sectional study includes 30 MF and 30 benign cutaneous inflammatory diseases (BCID) cases. All were evaluated using H & E and immunohistochemical staining for TOX & CD3. Results: There was statistically significant increase of TOX & CD3 expression in MF than BCID & ability of TOX to detect all true positive cases (100.0%) compared to (76.7%) for CD3, (P < 0.001). Conclusion: TOX had the highest sensitivity (100.0%) & accuracy (88.3%). TOX is useful marker in the diagnosis of early MF & differentiating it from BCID.

Diagnosis of Early Mycosis Fungoides.

Mycosis fungoides (MF), the most common type of cutaneous T-cell lymphomas, generally has a favorable clinical course. Early MF typically presents erythematous patches and/or plaques and lasts for many years without affecting the life expectancy. Only limited cases progress to develop skin tumors, with subsequent lymph nodes and rarely visceral organ involvement. One of the clinical problems in early MF is the difficulty in differentiating the disease from benign inflammatory disorders (BIDs), such as atopic dermatitis, chronic eczema, and psoriasis. In some MF cases, clinical and pathological findings are similar to those of BIDs. However, the accurate diagnosis of early MF is quite important, as inappropriate treatment including immunosuppressants can cause unfavorable or even fatal outcomes. This article focuses on general methods and novel tools for diagnosis of early MF.

A comparative study of non-Hodgkin lymphoma between public versus private institution in a middle-income country: A report of 2,317 cases.

e19569 Background: Non-Hodgkin lymphoma (NHL) is the most frequent hematological malignancy. B-cell (BCL) and T-cell (TCL) NHL subtypes differs in high income and low-and middle-income countries. In Peru, the healthcare system is divided mainly into public and private institutions. We aimed to describe the subtypes of NHL seen according to healthcare facilities. Methods: We reviewed medical records at National Cancer Institute and Oncosalud, both the leading public and private cancer centers in Peru, respectively. All patients diagnosed with NHL from 2015-2018 according to the 2016 WHO classification were included. Baseline characteristics were compared between public and private institutions using Student’s t test and Chi-square as appropriate. Results: A total of 2,317 NHL were included from both institutions. The median age was 61 years (range 15-99), 49.7% were male. Most patients NHL cases were encountered at the public institution (88.9%, n = 2,059); 84.4% (n = 1,957) were BCL and 15.5% (n = 360) TCL. Differences of BCL and TCL frequencies were seen among institutions. More BCL cases were seen at the private institution (96.6%, n = 230 versus 83.9%, n = 1,727, respectively) whereas TCL were common in the public institution (16.1%, n = 332 versus 11.8%, n = 28, respectively) (p &lt; 0.035). The most frequent BCL was DLBCL with 70.0% (n = 1209) and 49.6% (n = 114) seen in public and private institutions, respectively (p &lt; 0.001). The second most frequent BCL was follicular lymphoma (FL) with 10.8% (n = 187) and 20.4% (n = 47) seen in the public and private institutions, respectively (p &lt; 0.001). Chronic lymphocytic lymphoma (CLL) and Burkitt lymphoma (BL) were most frequent in private institution (CLL 7%, n = 16 vs. 3.5%, n = 60, p = 0.017; BL 3.9%, n = 9 vs. 1.2%, n = 21, p = 0.004). The most frequent TCL was peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) (27.7%, n = 92) for the public institution and mycosis fungoides (MF) (39.3%, n = 11) for the private institution. The second most frequent TCL was natural killer/TCL (NKTCL) (22.6%, n = 75) for the public institution, and PTCL, NOS (17.9%, n = 5) for the private institution. There was significant difference in the number of NKTCL and MF cases seen during the study period among institutions (NKTCL public 22.6%, n = 75 vs. private 3.6%, n = 1, p = 0.033; MF public 15.1%, n = 50 vs. private 39.3%, n = 11, p = 0.003). The presence of extranodal involvement was more frequent in the public institution (52.3%, n = 1,049 vs. 35.3%, n = 84, p &lt; 0.001). Conclusions: The distribution of NHL differs according to the type of healthcare system in Peru. Extranodal involvement, TCL, DLBCL and NKTCL are more frequent in patients treated at the public cancer center than in private center. On the contrary BCL, FL, CLL, BL and MF are more frequent in private cancer center. Our institutions are currently building the largest registry of NHL patients diagnosed and treated in Peru.

Clinical characteristics and long-term outcomes of 97 Korean patients with mycosis fungoides.

Mycosis fungoides (MF) shows racial and regional differences in terms of clinical features. The availability of therapeutic options as well as drugs differs from one country to another. There are only limited data on the clinical characteristics and treatment experience of MF from South Korea. Medical records of 97 patients with MF were retrospectively analyzed to investigate clinical features, survivals, and prognostic factors. Assessment of prognostic variables was done using univariate Cox proportional hazard models. Median age at time of diagnosis was 45 years. The median time from onset of skin lesion to diagnosis of MF was 36 months with a median follow-up period of 96 months. A number of clinical variants of MF were observed. Treatment mainly consisted of narrow-band UVB, systemic retinoids, methotrexate, chemotherapy, and regional radiotherapy. Complete remission was observed in 78% of patients with records on their clinical course. About 12% experienced disease progression. No clinical prognostic factor apart from TNM staging was identified. Despite delay in diagnosis, most cases of MF in Korea were diagnosed in early stages. Prognosis of our patients was more favorable than those of other geographic regions as reported in previous studies. Good response to treatment, consisting mainly of phototherapy and radiation therapy, and relatively indolent clinical behavior of disease were observed in this homogeneous cohort of Korean patients with MF.

Primary cutaneous T-cell lymphomas in childhood and adolescence.

Primary cutaneous lymphomas are extranodal non-Hodgkin lymphomas of T- or B- cell origin, that predominantly affect older patients but have been reported in all age groups and as early as in the first years of life. Diagnosis of cutaneous lymphomas is challenging and requires high clinical suspicion and close collaboration between dermatologists, pediatric oncologists and pathologists. Skin involvement of non-Hodgkin lymphomas in children or adolescents can either be primary cutaneous or secondary due to an underlying nodal lymphoma. The most common primary cutaneous lymphomas encountered in children are of T-cell origin, with mycosis fungoides being the most prevalent cutaneous T-cell lymphoma, followed by CD30+ lymphoproliferative disorders. While cutaneous lymphomas share clinicopathologic characteristics between juvenile and adult forms, there are important differences in terms of clinical presentation, diagnosis and treatment. The hypopigmented variant of mycosis fungoides seems to be overrepresented in the pediatric age group. Prognosis and treatment of mycosis fungoides are stage dependent. The majority of children present with early-stage disease and respond well to topical corticosteroids and phototherapy.

Clinicoprognostic implications of head and neck involvement by mycosis fungoides: A retrospective cohort study

The clinicoprognostic implications of head and neck involvement of mycosis fungoides (MF) are poorly understood. To evaluate the association of head and neck involvement on the clinicoprognostic features of MF. The clinical features and survival outcomes of patients with MF in a Korean academic medical center database were retrospectively evaluated according to the presence of head and neck involvement at diagnosis. Cases of MF with (group A, n=39) and without (group B, n=85) head and neck involvement at diagnosis were identified. Advanced-stage disease (stages IIB-IVB) was more common in group A (43.6%) than in group B (5.9%) (P<.001). MF progression, extracutaneous dissemination, and large-cell transformation more commonly occurred in group A than in group B. The 10-year overall survival rate was worse in group A (53.4%) compared with group B (81.6%) (P<.001). Head and neck involvement at diagnosis was associated with poor prognosis in early-stage MF (stages IA-IIA) and was independently associated with worse progression-free survival (hazard ratio, 24.4; 95% confidence interval, 2.2-267.6; P=.009). A single center, retrospective design. Head and neck involvement of MF was associated with a poor prognosis.

Mycosis fungoides and S\xe9zary syndrome: a population-wide study on prevalence and health care use in Finland in 1998\u20132016

BackgroundInformation about health care use and costs of cutaneous T-cell lymphoma (CTCL) patients is limited, particularly in a European setting.MethodsIn this population-wide study we set out to investigate prevalence, and trends in health care use in two CTCL subtypes, mycosis fungoides (MF) and Sézary syndrome (SS) over a time period of 19 years in 1998–2016 by using a nation-wide patient register containing data on all diagnosed MF and SS cases in Finland.ResultsThe prevalence of diagnosed MF and SS rose from 2.04 to 5.38/100000, and from 0.16 to 0.36/100000 for MF and SS respectively during 1998–2016. We found a substantial decrease in inpatient treatment of MF/SS in the past two decades with a mean of 2 inpatient days/patient/year due to MF/SS in 2016, while the mean numbers of MF/SS related outpatient visits remained stable at 8 visits/year/patient. Most MF/SS-related outpatient visits occurred in the medical specialty of dermatology. In a ten-year follow-up after MF/SS diagnosis, the main causes for outpatient visits and inpatient stays were MF/SS itself, other cancers, and other skin conditions. Also cardiovascular disease and infections contributed to the number of inpatient days. Mean total hospital costs decreased from 11,600 eur/patient/year to 3600 eur/patient/year by year 4 of the follow-up, and remained at that level for the remainder of the 10-year follow-up. MF/SS accounted for approximately half of the hospital costs of these patients throughout the follow-up.ConclusionsThe nearly 3-fold increase in prevalence of diagnosed MF/SS during 1998–2016 puts pressure on the health care system, as this is a high-cost patient group with a heavy burden of comorbidities. The challenge can be in part answered by shifting the treatment of MF/SS to a more outpatient-based practice, and by adapting new pharmacotherapy, as has been done in Finland.

The Role of Tumor Microenvironment in Mycosis Fungoides and Sézary Syndrome.

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphomas (CTCLs). Most cases of MF display an indolent course during its early stage. However, in some patients, it can progress to the tumor stage with potential systematic involvement and a poor prognosis. SS is defined as an erythrodermic CTCL with leukemic involvements. The pathogenesis of MF and SS is still not fully understood, but recent data have found that the development of MF and SS is related to genetic alterations and possibly to environmental influences. In CTCL, many components interacting with tumor cells, such as tumor-associated macrophages, fibroblasts, dendritic cells, mast cells, and myeloid-derived suppressor cells, as well as with chemokines, cytokines and other key players, establish the tumor microenvironment (TME). In turn, the TME regulates tumor cell migration and proliferation directly and indirectly and may play a critical role in the progression of MF and SS. The TME of MF and SS appear to show features of a Th2 phenotype, thus dampening tumor-related immune responses. Recently, several studies have been published on the immunological characteristics of MF and SS, but a full understanding of the CTCL-related TME remains to be determined. This review focuses on the role of the TME in MF and SS, aiming to further demonstrate the pathogenesis of the disease and to provide new ideas for potential treatments targeted at the microenvironment components of the tumor.

Two cases of mycosis fungoides diagnosed after treatment non-response to dupilumab

Two cases of mycosis fungoides diagnosed after treatment non-response to dupilumab

Management of Mycosis Fungoides with Topical Chlormethine/Mechlorethamine Gel: A Columbia University Cutaneous Lymphoma Center Experience.

Mycosis fungoides is a type of cutaneous T-cell lymphoma, which accounts for the majority of cases of cutaneous T-cell lymphoma. Mycosis fungoides can be classified as early-stage (IA–IIA) or late-stage (IIB or greater) disease. In early-stage mycosis fungoides, skin-directed therapies are commonly used to manage the disease. Chlormethine, or mechlorethamine, is a topical chemotherapeutic, which has been in use for over 60 years. In 2013, the US Food and Drug Administration approved chlormethine/mechlorethamine gel (Valchlor®) for treatment of stage IA and IB mycosis fungoides. Chlormethine/mechlorethamine gel is an effective therapy; however, its use may be limited by the development of adverse cutaneous reactions. Off-label dosing modifications, as well as co-administration of topical steroids and an aggressive moisturization regimen, can be used to reduce these side-effects. We report here 4 cases of mycosis fungoides treated with chlormethine/mechlorethamine gel at the Comprehensive Skin Cancer Center at Columbia University Irving Medical Center, which provide insights into the use of this therapy in clinical practice.

Mycosis fungoides and metabolic syndrome: A hospital-based case-control study

Background Emerging evidence now suggests an association between Mycosis fungoides (MF) and metabolic risk factors. Objective To investigate a possible association between MF and metabolic syndrome (MetS). Patients and methods This study was designed as an observational, case-control study. It included thirty MF patients and 39 controls who were assessed for body mass index (BMI), waist circumference, blood pressure, fasting blood glucose, triglycerides (TG), total cholesterol, high and low density lipoproteins, serum leptin level and insulin resistance. Results Our results revealed that serum leptin level (P=0.029), TG (P=0.004), cholesterol (P=0.001) and LDL (P=0.019), waist circumference in males (P Conclusion Our results indicate that patients with MF may be at higher risk for metabolic syndrome. Concurrently, screening for metabolic syndrome is recommended for all MF cases for early detection of cardiovascular risk.

Illness perception, coping, and quality of life in early-stage Mycosis fungoides

BackgroundMycosis fungoides is the most common type of cutaneous T-cell lymphoma. Most early-stage mycosis fungoides cases follow an indolent course, hence considered by doctors a relatively easy condition. However, since mycosis fungoides bears the title of cancer, patients might perceive it differently. ObjectiveTo investigate patients’ illness perception, and its relationships to quality of life, depression, anxiety, and coping among early-stage mycosis fungoides patients. MethodsA cross-sectional questionnaire-based study was conducted. Patients from a single tertiary medical center completed the Revised Illness Perception Questionnaire, the MF/SS-CTCL Quality of Life scale, the Hospital Anxiety and Depression Scale, and The Mental Adjustment to Cancer Scale. ResultsThirty patients (25 males, five females, mean age 51.60) with stage I mycosis fungoides were enrolled. Mycosis fungoides had a little impact on patients’ daily life, quality of life, and levels of depression and anxiety, and they generally coped well. Disease understanding was low and was negatively correlated with impairment to quality of life and depression. Patients felt that stress and worry were features of the disease’s etiology. Study limitationsA small sample of patients was included. ConclusionPatients with early-stage mycosis fungoides adapt well to their disease. Psychological interventions should be aimed at improving patients coping style and enhancing illness understanding, in order to maintain high quality of life.

Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients.

Simple SummaryCutaneous T-cell lymphomas (CTCL) are rare malignant diseases. In this study we have compared the cutaneous lymphoma registries of different countries, which included information on at least 100 patients. The frequencies of each CTCL subtype were compared within and between continents. We found that the registries differed importantly in terms of size and quality. Some rare CTCL subtypes, such as NK/T-cell lymphoma or subcutaneous panniculitis-like lymphomas, were more frequent in Asian countries, while others were evenly distributed. We discuss possible reasons for this and provide suggestions on how to build future CTCL registries.Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of rare diseases. Many studies have reported on local epidemiology or geographic clustering, however we lack information from a global perspective. A systematic review and meta-analysis was conducted in Medline and the Cochrane Library based on a previously registered protocol and according to the preferred reporting of items for systematic reviews and meta-analyses (PRISMA). We selected publications that enrolled at least 100 patients with primary cutaneous lymphomas according to the current classifications. The relative frequencies (proportions) of subtypes were compared between studies and geographic regions in a meta-analysis. In total, 26 studies met our inclusion criteria, reporting on altogether 16,953 patients. Within primary cutaneous lymphomas, CTCL appeared to be 15% more frequent in Asian populations. Mycosis fungoides (MF) accounted for 62% of CTCL, with an important heterogeneity in frequencies between studies and continents. The proportion of Sézary syndrome (SS) was 3%, stable worldwide. Rare CTCL, such as NK/T-cell lymphoma or subcutaneous panniculitis-like lymphoma, were more frequent in Asian studies. This global meta-analysis of CTCL confirmed the predominance of CTCL among primary cutaneous lymphomas (83% on average) in the three analyzed continents, most of which were MF cases. It revealed the same proportions of SS across continents, and the heterogeneity of MF frequencies, suggesting the possible role of environmental factors in the pathophysiology of the latter. Registration number: CRD42020148295 (PROSPERO).

CD20-positive T cell lymphoma: clinicopathological features of five cases

Objective: To investigate the clinicopathological characteristics of the T cell lymphomas with CD20 expression, and to better understand this rare entity. Methods: Two-hundred cases of T-cell lymphoma diagnosed in the Department of Pathology of the Affiliated Hospital of Qingdao University from November 2016 to February 2020 were examined, and 5 cases of CD20-positive T-cell lymphomas were identified and included. Combined with clinical data and review of the literature, the clinicopathological characteristics of the disease were analyzed. Results: The five patients were all male, and had an average age of 56 years (range, 47 to 64 years). There were 2 cases of monomorphic epitheliotropic intestinal T-cell lymphoma, 2 cases of mycosis fungoides (1 case was plaque stage and the other was tumor stage) and 1 case of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract. Immunohistochemistry showed that all 5 cases expressed multiple T cell markers (CD3/CD4/CD5/CD7/CD8) and only one of B cell markers (CD20). Three of the 5 cases were negative for CD20 at the first diagnosis, while CD20 was diffusely positive on the second biopsy from the recurrence or progression of the disease, without expression of CD79a or PAX5. Epstein-Barr encoding region (EBER) in situ hybridization was negative in all 5 cases. T-cell receptor gene analysis showed monoclonal rearrangement of β or/and δ chains;Ig rearrangements were all polyclonal. None of the five patients were treated with rituximab, and 4 patients survived with disease and 1 patient survived without disease at the end of follow-up. Among them, the patient with mycosis fungoides at the cancerous stage has progressed rapidly and had poor quality of life. Conclusions: CD20-positive T-cell lymphoma is extremely rare. Its prognosis is closely related to the type of T-cell lymphoma, clinical stage and initial therapeutic effect. However, the expression of CD20 indicates the recurrence or progression of the disease, and the prognosis is relatively poor. When CD3 expression is absent in T-cell lymphoma, it is easy to be misdiagnosed as B-cell lymphoma. The combination of multiple immunohistochemical antibodies and molecular detection can improve the accuracy of diagnosis.

Mycosis Fungoides: A Case Report

Objectives: Mycosis fungoides (MF) is the most common group of cutaneous T-cell lymphomas. We reported this rare diagnosed case of MF in National Dermatology Center of Mongolia. Methods: Diagnosis was based on physical examination and laboratory testing. Bioethical permission for this research was given by the Biomedical Ethics Committee of Ministry of Health in Mongolia, May 23, 2017. Results: A 78-year-old woman presented for evaluation of a 7-year history of recurrent pruritic lesions that appeared as red patches and plaques on non-sun exposed areas. She was given the initial clinical diagnosis of psoriasis. Upon examination, the morphology, histopathology and immunohistochemistry indicated MF, IB stage. Conclusions: A reliable diagnosis of mycosis fungoides might be feasible only in conjunction with clinical, histopathological, and molecular criteria.