Materiały źródłowe

• #1 Primary cutaneous lymphoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/primary-cutaneous-lymphoma?lang=us

  Primary cutaneous lymphomas represent a group of extranodal non-Hodgkin lymphomas (T- or B-cell) primarily confined to the skin with no evidence of extracutaneous disease at the time of diagnosis (cf. secondary involvement of the skin). […] They account for up to a fifth of all primary extranodal forms of non-Hodgkin lymphoma. […] Among the primary cutaneous lymphomas, B-cell types represent ~25% and T-cell types account for ~75% of the cases.

• #2 T-Cell Lymphoma – Lymphoma Research Foundation

  https://www.lymphoma.org/understanding-lymphoma/aboutlymphoma/nhl/t-cell-lymphoma/

  T-cell lymphomas account for about seven percent of all NHLs in the United States according to the Surveillance, Epidemiology, and End Results program. […] Cutaneous T-Cell Lymphoma (CTCL) accounts for two to three percent of all NHL cases and usually affects adults. The term cutaneous T-cell lymphoma describes a group of typically indolent lymphomas that appear on, and are most often confined to, the skin. […] Szary syndrome is a less common form of CTCL that affects both the skin and blood. Most cases occur in adults over the age of 60 years. The most common symptoms are swollen lymph nodes and a red, very itchy rash that covers large portions of the body.

• #3 Cutaneous T-Cell Lymphoma, Mycosis Fungoides | 5-Minute Clinical Consult

  https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816085/all/Cutaneous_T_Cell_Lymphoma_Mycosis_Fungoides?q=Cancer

  Cutaneous T-cell lymphoma (CTCL) is a rare and typically indolent mature T-cell lymphoma presenting primarily in the skin. This disease involves overlap of the disciplines of dermatology, medical oncology, and radiation oncology. […] Median age at diagnosis is 55 to 60 years; however, it can occur in children and young adults. Male-to-female ratio = 2:1. Incidence is higher in African Americans. […] 0.6 cases per 100,000 per year. ~4% of all non-Hodgkin lymphoma cases.

• #4 Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33050643/

  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 Szary 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).

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

  https://www.mdpi.com/2072-6694/12/10/2921

  The aim of this study was to describe the epidemiology of CTCL from a global perspective by a systematic review of the literature and meta-analysis. We also aimed to compare the cohorts based on geographic regions to identify a potential spatial clustering of cases, and to analyze the variations of the frequencies of different subgroups of CTCL over time. […] In this systematic review and meta-analysis, we reported on the relative frequencies of each CTCL subtype as compared to the total of CTCL cases. We compared these between studies and within continents. A major finding was that CTCL within cutaneous lymphomas seemed to be 15% to 17% more frequent in Asian and South American countries as compared to Europe. […] Information on the incidence of CTCL could be derived from some of the studies. Based on selected states, in the USA the incidence was estimated to be 0.77–0.87/100,000 person-years, whereas others reported an incidence of 0.64/100,000 person-years. In Europe, the incidence was estimated to be 0.29–0.39/100,000 person-years, with a possible increase over time.

• #6 Trends in Incidence of Cutaneous T-Cell Lymphoma in the United States – The ASCO Post

  https://ascopost.com/news/september-2022/trends-in-incidence-of-cutaneous-t-cell-lymphoma-in-the-united-states/

  In a study reported in a research letter in JAMA Oncology, Cai et al found that the incidence of cutaneous T-cell lymphoma increased in the United States between 2000 and 2018. […] The overall cutaneous T-cell lymphoma incidence was 8.55 per million persons; incidence significantly increased over the study period, with an annual percent change (APC) of 0.61 (95% confidence interval [CI] = 0.121.10). […] The investigators stated, We observed an increased overall cutaneous T-cell lymphoma incidence between 2000 and 2018. These findings differ from previous North American studies, which suggested cutaneous T-cell lymphoma incidence stabilized after 1998, but are consistent with recent studies from Europe.

• #7 Incidence of cutaneous T-cell lymphoma up in the U.S.

  https://medicalxpress.com/news/2022-09-incidence-cutaneous-t-cell-lymphoma.html

  The overall incidence of cutaneous T-cell lymphoma (CTCL) increased in the United States from 2000 to 2018, according to a research letter published online Sept. 1 in JAMA Oncology. […] From 2000 to 2018, the researchers identified 14,942 new cases of CTCL. The most common diagnosis was mycosis fungoides (MF), followed by primary CTCL (PCTCL) and primary cutaneous anaplastic large cell lymphoma. The overall incidence of CTCL was 8.55 per million, with an increase seen during the study period (annual percent change [APC], 0.61 percent). […] The highest incidence of CTCL was seen in men, non-Hispanic Black patients, individuals in the highest socioeconomic status quintiles, and those living in metropolitan counties (10.06, 11.68, 10.31, and 8.96, respectively). […] The overall incidence rate was six times higher for patients aged 40 years or older versus those aged younger than 40 years; however, patients aged younger than 40 years had significantly higher increases in overall CTCL and MF incidence rates (APC, 2.87 and 3.67 percent, respectively). […] „One key new finding is the more rapid increase in incidence among young patients, possibly owing to earlier diagnosis,” the authors write.

• #8 RESEARCH DISCOVERS CTCL INCIDENCE RISING IN United States | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/research-discovers-ctcl-incidence-rising-united-states

  The Cutaneous Lymphoma Foundation has funded an important epidemiology study that shows that the number of new cases of CTCL has doubled. In this study, published in the July 2007 issue of the Archives of Dermatology, Drs. Vincent Criscione and Martin Weinstock of Brown University report that there are approximately 1,600 new cases of CTCL diagnosed each year in the United States. This represents over double the number of new cases in the past fifteen years. […] They determined that the overall annual incidence of CTCL was 6.4 per million, a total of 0.14 percent of all cancers, and 3.9 percent of non-Hodgkins lymphomas. This represents a more than doubling of the incidence since the last study that was conducted in 1992. […] The exact cause for the observed increase in CTCL incidence is unknown and cannot be determined from this epidemiology study. […] Future studies are needed to determine death rate, prevalence and the impact of treatment on CTCL survival.

• #9 RESEARCH DISCOVERS CTCL INCIDENCE RISING IN United States | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/research-discovers-ctcl-incidence-rising-united-states

  The Cutaneous Lymphoma Foundation has funded an important epidemiology study that shows that the number of new cases of CTCL has doubled. In this study, published in the July 2007 issue of the Archives of Dermatology, Drs. Vincent Criscione and Martin Weinstock of Brown University report that there are approximately 1,600 new cases of CTCL diagnosed each year in the United States. This represents over double the number of new cases in the past fifteen years. […] They determined that the overall annual incidence of CTCL was 6.4 per million, a total of 0.14 percent of all cancers, and 3.9 percent of non-Hodgkins lymphomas. This represents a more than doubling of the incidence since the last study that was conducted in 1992. […] The exact cause for the observed increase in CTCL incidence is unknown and cannot be determined from this epidemiology study. […] Future studies are needed to determine death rate, prevalence and the impact of treatment on CTCL survival.

• #10 Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33050643/

  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 Szary 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).

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

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7600606/

  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. […] The aim of this study was to describe the epidemiology of CTCL from a global perspective by a systematic review of the literature and meta-analysis. We also aimed to compare the cohorts based on geographic regions to identify a potential spatial clustering of cases, and to analyze the variations of the frequencies of different subgroups of CTCL over time. […] In this systematic review and meta-analysis, we reported on the relative frequencies of each CTCL subtype as compared to the total of CTCL cases. We compared these between studies and within continents. A major finding was that CTCL within cutaneous lymphomas seemed to be 15% to 17% more frequent in Asian and South American countries as compared to Europe.

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

  https://www.mdpi.com/2072-6694/12/10/2921

  The aim of this study was to describe the epidemiology of CTCL from a global perspective by a systematic review of the literature and meta-analysis. We also aimed to compare the cohorts based on geographic regions to identify a potential spatial clustering of cases, and to analyze the variations of the frequencies of different subgroups of CTCL over time. […] In this systematic review and meta-analysis, we reported on the relative frequencies of each CTCL subtype as compared to the total of CTCL cases. We compared these between studies and within continents. A major finding was that CTCL within cutaneous lymphomas seemed to be 15% to 17% more frequent in Asian and South American countries as compared to Europe. […] Information on the incidence of CTCL could be derived from some of the studies. Based on selected states, in the USA the incidence was estimated to be 0.77–0.87/100,000 person-years, whereas others reported an incidence of 0.64/100,000 person-years. In Europe, the incidence was estimated to be 0.29–0.39/100,000 person-years, with a possible increase over time.

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

  https://www.mdpi.com/2072-6694/12/10/2921

  Epidemiology of Cutaneous 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. […] 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.

• #14 Mycosis Fungoides : Epidemiology

  https://www.webpathology.com/images/hematopathology/mature-t-cell-and-nk-cell-neoplasms/mycosis-fungoides-and-sezary-syndrome/38062

  Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma and also makes up almost 50% of all primary cutaneous lymphomas. Most cases affect adult and elderly patients (median age at diagnosis 55-60 years), but it can also be seen in adolescents and young adults. There is male predominance with M:F ratio of 2:1. It is slightly more common in blacks and is seen less often in Asians and Hispanics. Higher risk of MF has been associated with certain professions/industries, including farming, painting, woodworking, carpentry, textiles, petrochemicals and metal industries.

• #15 Cutaneous T-Cell Lymphoma, Mycosis Fungoides | 5-Minute Clinical Consult

  https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816085/all/Cutaneous_T_Cell_Lymphoma_Mycosis_Fungoides?q=Cancer

  Cutaneous T-cell lymphoma (CTCL) is a rare and typically indolent mature T-cell lymphoma presenting primarily in the skin. This disease involves overlap of the disciplines of dermatology, medical oncology, and radiation oncology. […] Median age at diagnosis is 55 to 60 years; however, it can occur in children and young adults. Male-to-female ratio = 2:1. Incidence is higher in African Americans. […] 0.6 cases per 100,000 per year. ~4% of all non-Hodgkin lymphoma cases.

• #16 Cutaneous T-Cell Lymphoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/2139720-overview

  Cutaneous T-cell lymphoma has a sex predilection, being more common in men than women by a ratio of approximately 2:1. […] Most patients with cutaneous T-cell lymphoma are middle-aged or elderly. Szary syndrome, for example, occurs almost exclusively in adults. Many patients have had a poorly defined form of dermatitis for many years prior to the onset of the disease. In a significant proportion of cases, the onset of the disease, or of a dermatitic precursor of the disease, occurs in childhood. […] However, cutaneous T-cell lymphoma is exceedingly rare in children younger than 10 years, and in such cases it does not show a male predominance; one series even reported a strong female predilection. Similar to adult patients, most children present in stage IA or IB and have a good to excellent prognosis with treatment, although cases progressing to plaque, tumor-stage disease, and death have been reported.

• #17 Cutaneous T-Cell Lymphoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/2139720-overview

  Cutaneous T-cell lymphoma has a sex predilection, being more common in men than women by a ratio of approximately 2:1. […] Most patients with cutaneous T-cell lymphoma are middle-aged or elderly. Szary syndrome, for example, occurs almost exclusively in adults. Many patients have had a poorly defined form of dermatitis for many years prior to the onset of the disease. In a significant proportion of cases, the onset of the disease, or of a dermatitic precursor of the disease, occurs in childhood. […] However, cutaneous T-cell lymphoma is exceedingly rare in children younger than 10 years, and in such cases it does not show a male predominance; one series even reported a strong female predilection. Similar to adult patients, most children present in stage IA or IB and have a good to excellent prognosis with treatment, although cases progressing to plaque, tumor-stage disease, and death have been reported.

• #18 Cutaneous T-cell lymphoma – Wikipedia

  https://en.wikipedia.org/wiki/Cutaneous_T-cell_lymphoma

  Of all cancers involving lymphocytes, 2% of cases are cutaneous T cell lymphomas. CTCL is more common in men and in African-American people. The incidence of CTCL in men is 1.6 times higher than in women. […] There is some evidence of a relationship with human T-lymphotropic virus (HTLV) with the adult T-cell leukemia/lymphoma subtype. No definitive link between any viral infection or environmental factor has been definitely shown with other CTCL subtypes.

• #19 Cutaneous T-Cell Lymphoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/2139720-overview

  Cutaneous T-cell lymphoma has a worldwide distribution; some studies have identified a higher prevalence of the disease in industrial populations (eg, among workers who use machine cutting oils). The incidence of cutaneous T-cell lymphoma in the United States was reported to be 6.4 persons per million population annually (for the overall, age-adjusted incidence) between 1973 and 2002, with the diseases incidence increasing over that time period. […] Adult T-cell lymphoma/leukemia is endemic in areas with a high prevalence of HTLV-1 infection, such as southwest Japan, the Caribbean islands, South America, and parts of central Africa. This disease occurs in 1-5% of seropositive individuals after more than 2 decades of viral persistence. […] In the United States, cutaneous T-cell lymphoma is more common among persons of sub-Saharan African lineage than among those of European background, in a ratio of approximately 2:1. In Kuwait, a study found that the annual incidence rate of mycosis fungoides was significantly higher among Arabs than among non-Arab Asians.

• #20 Incidence of cutaneous T-cell lymphoma up in the U.S.

  https://medicalxpress.com/news/2022-09-incidence-cutaneous-t-cell-lymphoma.html

  The overall incidence of cutaneous T-cell lymphoma (CTCL) increased in the United States from 2000 to 2018, according to a research letter published online Sept. 1 in JAMA Oncology. […] From 2000 to 2018, the researchers identified 14,942 new cases of CTCL. The most common diagnosis was mycosis fungoides (MF), followed by primary CTCL (PCTCL) and primary cutaneous anaplastic large cell lymphoma. The overall incidence of CTCL was 8.55 per million, with an increase seen during the study period (annual percent change [APC], 0.61 percent). […] The highest incidence of CTCL was seen in men, non-Hispanic Black patients, individuals in the highest socioeconomic status quintiles, and those living in metropolitan counties (10.06, 11.68, 10.31, and 8.96, respectively). […] The overall incidence rate was six times higher for patients aged 40 years or older versus those aged younger than 40 years; however, patients aged younger than 40 years had significantly higher increases in overall CTCL and MF incidence rates (APC, 2.87 and 3.67 percent, respectively). […] „One key new finding is the more rapid increase in incidence among young patients, possibly owing to earlier diagnosis,” the authors write.

• #21 Incidence of cutaneous T-cell lymphoma up in the U.S.

  https://medicalxpress.com/news/2022-09-incidence-cutaneous-t-cell-lymphoma.html

  The overall incidence of cutaneous T-cell lymphoma (CTCL) increased in the United States from 2000 to 2018, according to a research letter published online Sept. 1 in JAMA Oncology. […] From 2000 to 2018, the researchers identified 14,942 new cases of CTCL. The most common diagnosis was mycosis fungoides (MF), followed by primary CTCL (PCTCL) and primary cutaneous anaplastic large cell lymphoma. The overall incidence of CTCL was 8.55 per million, with an increase seen during the study period (annual percent change [APC], 0.61 percent). […] The highest incidence of CTCL was seen in men, non-Hispanic Black patients, individuals in the highest socioeconomic status quintiles, and those living in metropolitan counties (10.06, 11.68, 10.31, and 8.96, respectively). […] The overall incidence rate was six times higher for patients aged 40 years or older versus those aged younger than 40 years; however, patients aged younger than 40 years had significantly higher increases in overall CTCL and MF incidence rates (APC, 2.87 and 3.67 percent, respectively). […] „One key new finding is the more rapid increase in incidence among young patients, possibly owing to earlier diagnosis,” the authors write.

• #22 Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33050643/

  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 Szary 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).

• #23 Mycosis fungoides

  https://dermnetnz.org/topics/mycosis-fungoides

  Mycosis fungoides (MF) represents 50% of all primary cutaneous lymphomas and 60-70% of all cutaneous T-cell lymphomas (CTCL). However, it is an uncommon condition with an estimated incidence worldwide of 6.4 per million. […] Mycosis fungoides is the most common primary cutaneous lymphoma (65%) in children but diagnosis is often delayed (25 years). The mean age at diagnosis is 10 years, and the sexes are affected almost equally. Most children present with non-classic and often multiple variants. More than 50% of children present with the hypopigmented variant of MF; classic MF affects 40%, and folliculotropic MF in one-third. […] A large case series from Johns Hopkins reported Black American males presented with later stage disease and greater skin involvement than white Americans; Black American females had early-onset aggressive disease. Patients with hypopigmented lesions were more likely to have early-stage disease and better survival than those with advanced-stage disease in another US series.

• #24 Mycosis fungoides

  https://dermnetnz.org/topics/mycosis-fungoides

  Mycosis fungoides (MF) represents 50% of all primary cutaneous lymphomas and 60-70% of all cutaneous T-cell lymphomas (CTCL). However, it is an uncommon condition with an estimated incidence worldwide of 6.4 per million. […] Mycosis fungoides is the most common primary cutaneous lymphoma (65%) in children but diagnosis is often delayed (25 years). The mean age at diagnosis is 10 years, and the sexes are affected almost equally. Most children present with non-classic and often multiple variants. More than 50% of children present with the hypopigmented variant of MF; classic MF affects 40%, and folliculotropic MF in one-third. […] A large case series from Johns Hopkins reported Black American males presented with later stage disease and greater skin involvement than white Americans; Black American females had early-onset aggressive disease. Patients with hypopigmented lesions were more likely to have early-stage disease and better survival than those with advanced-stage disease in another US series.

• #25 Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33050643/

  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 Szary 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).

• #26 Early-stage Mycosis Fungoides: Epidemiology and Prognosis | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3367

  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 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. […] We confirm the rarity of early-stage mycosis fungoides, a differential prognosis and the potential for elevated burden of disease. […] However, prevalence of early-stage disease is unknown. […] The cancer registries provided 3,132 early-stage mycosis fungoides patients. […] This confirms the rarity of early-stage mycosis fungoides and a differential prognosis in early-stage.

• #27 Early-stage Mycosis Fungoides: Epidemiology and Prognosis | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3367

  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 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. […] We confirm the rarity of early-stage mycosis fungoides, a differential prognosis and the potential for elevated burden of disease. […] However, prevalence of early-stage disease is unknown. […] The cancer registries provided 3,132 early-stage mycosis fungoides patients. […] This confirms the rarity of early-stage mycosis fungoides and a differential prognosis in early-stage.

• #28 T-Cell Lymphoma – Lymphoma Research Foundation

  https://lymphoma.org/understanding-lymphoma/aboutlymphoma/nhl/t-cell-lymphoma/

  T-cell lymphomas account for about seven percent of all NHLs in the United States according to the Surveillance, Epidemiology, and End Results program. […] Cutaneous T-Cell Lymphoma (CTCL) accounts for two to three percent of all NHL cases and usually affects adults. The term cutaneous T-cell lymphoma describes a group of typically indolent lymphomas that appear on, and are most often confined to, the skin. […] Mycosis fungoides, which appears as skin patches, plaques, or tumors, is the most common type of CTCL. […] Szary syndrome is a less common form of CTCL that affects both the skin and blood. Most cases occur in adults over the age of 60 years. The most common symptoms are swollen lymph nodes and a red, very itchy rash that covers large portions of the body. […] Other, rare forms of CTCL include primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis.

• #29 Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33050643/

  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 Szary 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).

• #30 Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33050643/

  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 Szary 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).

• #31 CTCL Is Rare, Incurable, and Potentially Lethal in Advanced Stages

  https://www.onclive.com/view/ctcl-is-rare-incurable-and-potentially-lethal-in-advanced-stages

  Although cutaneous T-cell lymphoma, a group of non-Hodgkin T-cell lymphomas, remains a rare disease, incidence has increased steadily since the 1970s. […] As reported by the Surveillance, Epidemiology and End Results (SEER) registry data, annual overall incidence in the United States was 6.4 cases per million persons from 1973 to 2002, increasing to 7.7 per million persons in 2001 to 2005. […] Large-scale SEER and National Cancer Database studies showed that Black/African American patients have a higher incidence rate, younger age of onset, higher disease burden, and inferior survival compared with their White counterparts. […] The predicted 10-year overall survival (OS) rate is 90.3% for early-stage disease vs 53.2% for advanced stage disease. […] In findings from a retrospective cross-sectional observational study of 102 patients with stage IA and stage IB MF, investigators observed disease progression (upstaging) in 29.4% of patients.

• #32 Trends in Incidence of Cutaneous T-Cell Lymphoma in the United States – The ASCO Post

  https://ascopost.com/news/september-2022/trends-in-incidence-of-cutaneous-t-cell-lymphoma-in-the-united-states/

  In a study reported in a research letter in JAMA Oncology, Cai et al found that the incidence of cutaneous T-cell lymphoma increased in the United States between 2000 and 2018. […] The overall cutaneous T-cell lymphoma incidence was 8.55 per million persons; incidence significantly increased over the study period, with an annual percent change (APC) of 0.61 (95% confidence interval [CI] = 0.121.10). […] The investigators stated, We observed an increased overall cutaneous T-cell lymphoma incidence between 2000 and 2018. These findings differ from previous North American studies, which suggested cutaneous T-cell lymphoma incidence stabilized after 1998, but are consistent with recent studies from Europe.

• #33 Incidence of cutaneous T-cell lymphoma up in the U.S.

  https://medicalxpress.com/news/2022-09-incidence-cutaneous-t-cell-lymphoma.html

  The overall incidence of cutaneous T-cell lymphoma (CTCL) increased in the United States from 2000 to 2018, according to a research letter published online Sept. 1 in JAMA Oncology. […] From 2000 to 2018, the researchers identified 14,942 new cases of CTCL. The most common diagnosis was mycosis fungoides (MF), followed by primary CTCL (PCTCL) and primary cutaneous anaplastic large cell lymphoma. The overall incidence of CTCL was 8.55 per million, with an increase seen during the study period (annual percent change [APC], 0.61 percent). […] The highest incidence of CTCL was seen in men, non-Hispanic Black patients, individuals in the highest socioeconomic status quintiles, and those living in metropolitan counties (10.06, 11.68, 10.31, and 8.96, respectively). […] The overall incidence rate was six times higher for patients aged 40 years or older versus those aged younger than 40 years; however, patients aged younger than 40 years had significantly higher increases in overall CTCL and MF incidence rates (APC, 2.87 and 3.67 percent, respectively). […] „One key new finding is the more rapid increase in incidence among young patients, possibly owing to earlier diagnosis,” the authors write.

• #34 Trends in Incidence of Cutaneous T-Cell Lymphoma in the United States – The ASCO Post

  https://ascopost.com/news/september-2022/trends-in-incidence-of-cutaneous-t-cell-lymphoma-in-the-united-states/

  In a study reported in a research letter in JAMA Oncology, Cai et al found that the incidence of cutaneous T-cell lymphoma increased in the United States between 2000 and 2018. […] The overall cutaneous T-cell lymphoma incidence was 8.55 per million persons; incidence significantly increased over the study period, with an annual percent change (APC) of 0.61 (95% confidence interval [CI] = 0.121.10). […] The investigators stated, We observed an increased overall cutaneous T-cell lymphoma incidence between 2000 and 2018. These findings differ from previous North American studies, which suggested cutaneous T-cell lymphoma incidence stabilized after 1998, but are consistent with recent studies from Europe.

• #35 Incidence of Cutaneous T-Cell Lymphoma Up in the U.S. – Southern Iowa Mental Health Center

  https://simhcottumwa.org/incidence-of-cutaneous-t-cell-lymphoma-up-in-the-u-s/

  Incidence of Cutaneous T-Cell Lymphoma Up in the U.S. […] The overall incidence of cutaneous T-cell lymphoma (CTCL) increased in the United States from 2000 to 2018, according to a research letter published online Sept. 1 in JAMA Oncology. […] From 2000 to 2018, the researchers identified 14,942 new cases of CTCL. […] The overall incidence of CTCL was 8.55 per million, with an increase seen during the study period (annual percent change [APC], 0.61 percent). […] The highest incidence of CTCL was seen in men, non-Hispanic Black patients, individuals in the highest socioeconomic status quintiles, and those living in metropolitan counties (10.06, 11.68, 10.31, and 8.96, respectively). […] The overall incidence rate was six times higher for patients aged 40 years or older versus those aged younger than 40 years; however, patients aged younger than 40 years had significantly higher increases in overall CTCL and MF incidence rates (APC, 2.87 and 3.67 percent, respectively). […] „One key new finding is the more rapid increase in incidence among young patients, possibly owing to earlier diagnosis,” the authors write.

• #36 Cutaneous T-cell lymphoma – Wikipedia

  https://en.wikipedia.org/wiki/Cutaneous_T-cell_lymphoma

  Of all cancers involving lymphocytes, 2% of cases are cutaneous T cell lymphomas. CTCL is more common in men and in African-American people. The incidence of CTCL in men is 1.6 times higher than in women. […] There is some evidence of a relationship with human T-lymphotropic virus (HTLV) with the adult T-cell leukemia/lymphoma subtype. No definitive link between any viral infection or environmental factor has been definitely shown with other CTCL subtypes.

• #37 Cutaneous T-Cell Lymphoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/2139720-overview

  Cutaneous T-cell lymphoma has a worldwide distribution; some studies have identified a higher prevalence of the disease in industrial populations (eg, among workers who use machine cutting oils). The incidence of cutaneous T-cell lymphoma in the United States was reported to be 6.4 persons per million population annually (for the overall, age-adjusted incidence) between 1973 and 2002, with the diseases incidence increasing over that time period. […] Adult T-cell lymphoma/leukemia is endemic in areas with a high prevalence of HTLV-1 infection, such as southwest Japan, the Caribbean islands, South America, and parts of central Africa. This disease occurs in 1-5% of seropositive individuals after more than 2 decades of viral persistence. […] In the United States, cutaneous T-cell lymphoma is more common among persons of sub-Saharan African lineage than among those of European background, in a ratio of approximately 2:1. In Kuwait, a study found that the annual incidence rate of mycosis fungoides was significantly higher among Arabs than among non-Arab Asians.

• #38 Mycosis Fungoides : Epidemiology

  https://www.webpathology.com/images/hematopathology/mature-t-cell-and-nk-cell-neoplasms/mycosis-fungoides-and-sezary-syndrome/38062

  Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma and also makes up almost 50% of all primary cutaneous lymphomas. Most cases affect adult and elderly patients (median age at diagnosis 55-60 years), but it can also be seen in adolescents and young adults. There is male predominance with M:F ratio of 2:1. It is slightly more common in blacks and is seen less often in Asians and Hispanics. Higher risk of MF has been associated with certain professions/industries, including farming, painting, woodworking, carpentry, textiles, petrochemicals and metal industries.

• #39 The Incidence of Cutaneous T-Cell Lymphoma Seems to Be Higher in Veterans | CheckRare

  https://checkrare.com/the-incidence-of-cutaneous-t-cell-lymphoma-seems-to-be-higher-in-veterans/

  According to a paper published in U.S. Medicine, The Veterans Affairs (VA) classifies CTCL and other non-Hodgkin lymphomas as presumptively caused by Agent Orange exposure or service in the Vietnam theater, even without exposure to the problematic herbicide. Both arise at markedly elevated rates in veterans, but it had remained unknown just how much higher the rate of CTCL was. […] In a study published in the Journal of Investigative Dermatology, researchers noted that an estimated 5-15% of all CTCL diagnosed each year occurs in veterans, a percentage far greater than the percentage of veterans in the total U.S. population. […] Researchers found a 6 to 8 times higher incidence of CTCL in veterans than in the general population. […] The findings suggest that specific military exposures, such as Agent Orange during the Vietnam War, may be potential pathogenic drivers and warrant further exploration of the causes behind increased incidence of CTCL in veterans.

• #40 Skin (cutaneous) T-cell lymphoma | Lymphoma Action

  https://lymphoma-action.org.uk/types-lymphoma-skin-lymphoma/skin-cutaneous-t-cell-lymphoma

  T-cell skin lymphoma (also called cutaneous T-cell lymphoma, or CTCL) is rare. Fewer than 350 people are diagnosed with T-cell skin lymphoma in the UK each year. […] Scientists dont know exactly what causes skin lymphoma but there are some factors that might increase your risk of developing it. […] T-cell skin lymphoma is more common in older people. The average age at diagnosis is 55. […] Men are around twice as likely to develop T-cell skin lymphoma as women. […] Research in the United States has found that T-cell skin lymphoma is more common in African American people than it is in white people. […] T-cell skin lymphoma is more common in people who have had a transplant (post-transplant lymphoproliferative disorder, or PTLD) and in people who have HIV. […] It is important to remember that T-cell skin lymphomas are rare.

• #41

  https://step2.medbullets.com/oncology/120392/cutaneous-t-cell-lymphoma

  Epidemiology […] Older patients (median age 55-60) […] More common in male patients […] More common in black patients […] […] […] Diagnosis usually made by clinical exam and history […] Diagnosis confirmed with skin biopsy […] […] […] Stage-dependent […] Mycosis fungoides typically indolent […] Szary syndrome typically aggressive […] can be fatal

• #42 Mycosis fungoides

  https://dermnetnz.org/topics/mycosis-fungoides

  The diagnosis of mycosis fungoides requires careful clinicopathological correlation. Dermoscopy can help to distinguish MF from inflammatory dermatosis, other causes of erythroderma, and leprosy. […] Mycosis fungoides typically follows an indolent course over years, with an estimated 5-year survival of 87%. The duration of early-stage disease has been estimated to be approximately 18 years. Although improved survival with MF has been demonstrated, it is likely due to earlier diagnosis rather than advances in therapy.

• #43 CTCL Is Rare, Incurable, and Potentially Lethal in Advanced Stages

  https://www.onclive.com/view/ctcl-is-rare-incurable-and-potentially-lethal-in-advanced-stages

  Although cutaneous T-cell lymphoma, a group of non-Hodgkin T-cell lymphomas, remains a rare disease, incidence has increased steadily since the 1970s. […] As reported by the Surveillance, Epidemiology and End Results (SEER) registry data, annual overall incidence in the United States was 6.4 cases per million persons from 1973 to 2002, increasing to 7.7 per million persons in 2001 to 2005. […] Large-scale SEER and National Cancer Database studies showed that Black/African American patients have a higher incidence rate, younger age of onset, higher disease burden, and inferior survival compared with their White counterparts. […] The predicted 10-year overall survival (OS) rate is 90.3% for early-stage disease vs 53.2% for advanced stage disease. […] In findings from a retrospective cross-sectional observational study of 102 patients with stage IA and stage IB MF, investigators observed disease progression (upstaging) in 29.4% of patients.

• #44 CTCL Is Rare, Incurable, and Potentially Lethal in Advanced Stages

  https://www.onclive.com/view/ctcl-is-rare-incurable-and-potentially-lethal-in-advanced-stages

  Although cutaneous T-cell lymphoma, a group of non-Hodgkin T-cell lymphomas, remains a rare disease, incidence has increased steadily since the 1970s. […] As reported by the Surveillance, Epidemiology and End Results (SEER) registry data, annual overall incidence in the United States was 6.4 cases per million persons from 1973 to 2002, increasing to 7.7 per million persons in 2001 to 2005. […] Large-scale SEER and National Cancer Database studies showed that Black/African American patients have a higher incidence rate, younger age of onset, higher disease burden, and inferior survival compared with their White counterparts. […] The predicted 10-year overall survival (OS) rate is 90.3% for early-stage disease vs 53.2% for advanced stage disease. […] In findings from a retrospective cross-sectional observational study of 102 patients with stage IA and stage IB MF, investigators observed disease progression (upstaging) in 29.4% of patients.

• #45

  https://www.jci.org/articles/view/24826

  Mycosis fungoides (MF) accounts for approximately 1% of all non-Hodgkin lymphomas, with a median age of presentation of 57 years and a male/female ratio of 2:1. Its incidence is estimated at 0.36-0.90 per 100,000 person-years. This rate appears to be stable, although cases of non-Hodgkin lymphoma overall appear to have doubled in the past 2 decades and likely MF/SS is underreported due to nomenclature variances. […] Several studies have demonstrated that early MF is typically indolent, and patients with limited patches or plaques who have received skin-directed treatment have median survival similar to that of matched control populations. Survival decreases at more advanced stages, and 10% of all patients will progress to more advanced disease. SS patients traditionally have had a poor prognosis, with only approximately 30% of patients surviving beyond 5 years after diagnosis. Important clinical prognostic indicators in MF/SS are T classification, clinical stage, presence of extracutaneous disease, and patient age. An elevated Szary count, elevated lactate dehydrogenase level, and peripheral eosinophilia also portend poorer prognosis.

• #46

  https://link.springer.com/article/10.1007/s13671-012-0038-2

  Over the past 3 years, survival data from two of the largest single-center series of MF/SS patients have been published using updated 2007 International Society for Cutaneous Lymphoma-European Organization for Research and Treatment of Cancer (ISCL/EORTC) staging criteria. Agar et al. from the UK analyzed their cohort of 1,502 MF/ SS followed from 1980-2009 for overall survival (OS), disease specific survival (DSS) and risk of disease progression (RDP). Seventy-one percent of these patients presented with early stage (Stage IA, IB, IIA) disease. Disease progression to a higher stage occurred in 34 % of patients; 25 % patients ultimately died of their disease. […] This year, Kubica et al. from the Mayo Clinic described 176 SS patients followed from 1976-2010 and reported a median OS of 4.0 years, which also seems to support a possible trend towards improved survival from the past.

• #47 CTCL Is Rare, Incurable, and Potentially Lethal in Advanced Stages

  https://www.onclive.com/view/ctcl-is-rare-incurable-and-potentially-lethal-in-advanced-stages

  Investigators found that stage IV disease, being older than 60 years of age, having large-cell transformation, and increased lactate dehydrogenase were independent prognostic markers for a worse survival. […] Chen noted that advanced-stage disease can be highly disfiguring and lethal and often fails to respond to multiple forms of systemic therapy.

• #48 Mycosis fungoides

  https://dermnetnz.org/topics/mycosis-fungoides

  The diagnosis of mycosis fungoides requires careful clinicopathological correlation. Dermoscopy can help to distinguish MF from inflammatory dermatosis, other causes of erythroderma, and leprosy. […] Mycosis fungoides typically follows an indolent course over years, with an estimated 5-year survival of 87%. The duration of early-stage disease has been estimated to be approximately 18 years. Although improved survival with MF has been demonstrated, it is likely due to earlier diagnosis rather than advances in therapy.

• #49 (PDF) Trends in Cutaneous Lymphoma Epidemiology

  https://www.academia.edu/18659167/Trends_in_Cutaneous_Lymphoma_Epidemiology

  Cutaneous T-cell lymphoma (CTCL) is a rare, heterogeneous group of non-Hodgkin lymphomas characterized by various clinical, molecular, and histopathologic features of the skin. Variants of CTCL share many clinical features with common inflammatory skin diseases such as atopic dermatitis and psoriasis, making accurate and early diagnosis challenging in clinical settings. […] The current incidence estimate of 40 traumatic spinal cord injuries (TSCI) per million population/year in the United States (U.S.) is based on data from the 1990s. […] The etiology of cutaneous T-cell lymphoma (CTCL) remains unknown, with potential infectious causes having been explored. […] The most common type of CTCL is mycosis fungoides (MF), which represents about 70% of all cases. […] Although 80% to 90% of those affected with early MF have a favorable prognosis, 10% to 20% can progress to more advanced disease with a marked increase in mortality (26% survival at 5 years).

• #50 Mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL) epidemiology and treatment pathway in Spain: new insights for an accurate description – Drugs in Context

  https://www.drugsincontext.com/mycosis-fungoides-type-cutaneous-t-cell-lymphoma-mf-ctcl-epidemiology-and-treatment-pathway-in-spain-new-insights-for-an-accurate-description/

  Mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL) is a rare lymphoma localized in the skin. Due to its indolent nature and similarity to other skin conditions, diagnosis is often delayed or incorrect. Consequently, accurate calculations of incidence and prevalence are difficult to make. The treatment pathway taken by MF-CTCL patients can differ depending upon local healthcare systems, clinical policies and guidelines. This study aims to (1) provide an estimate for the prevalence of treated MF-CTCL patients in Spain, (2) describe the Spanish patient treatment pathways for MF-CTCL, including quantification of the distribution of patients between primary, secondary and tertiary care institutions, and (3) investigate and quantify the treatment preferences of physicians. Poor diagnosis of MF-CTCL may mean that actual prevalence levels in the broader population are higher than those estimated by this analysis of treated patients. Around 75% of MF-CTCL patients in Spain report that the initial diagnosis by their general practitioner is incorrect. This is usually due to underestimation of severity or type of skin disease. Following diagnosis, the majority of patient management is conducted by secondary or tertiary care centers. Incidence rates have increased in recent years, and possible reasons for this include improving levels of diagnosis. However, it is still likely that there are further undiagnosed MF-CTCL patients in Spain due to the challenges of diagnosis at the primary care level. […] This trend appears to be reflected in the prevalence reported in this study, which is higher than suggested by some other estimates.

• #51 Mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL) epidemiology and treatment pathway in Spain: new insights for an accurate description – Drugs in Context

  https://www.drugsincontext.com/mycosis-fungoides-type-cutaneous-t-cell-lymphoma-mf-ctcl-epidemiology-and-treatment-pathway-in-spain-new-insights-for-an-accurate-description/

  Mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL) is a rare lymphoma localized in the skin. Due to its indolent nature and similarity to other skin conditions, diagnosis is often delayed or incorrect. Consequently, accurate calculations of incidence and prevalence are difficult to make. The treatment pathway taken by MF-CTCL patients can differ depending upon local healthcare systems, clinical policies and guidelines. This study aims to (1) provide an estimate for the prevalence of treated MF-CTCL patients in Spain, (2) describe the Spanish patient treatment pathways for MF-CTCL, including quantification of the distribution of patients between primary, secondary and tertiary care institutions, and (3) investigate and quantify the treatment preferences of physicians. Poor diagnosis of MF-CTCL may mean that actual prevalence levels in the broader population are higher than those estimated by this analysis of treated patients. Around 75% of MF-CTCL patients in Spain report that the initial diagnosis by their general practitioner is incorrect. This is usually due to underestimation of severity or type of skin disease. Following diagnosis, the majority of patient management is conducted by secondary or tertiary care centers. Incidence rates have increased in recent years, and possible reasons for this include improving levels of diagnosis. However, it is still likely that there are further undiagnosed MF-CTCL patients in Spain due to the challenges of diagnosis at the primary care level. […] This trend appears to be reflected in the prevalence reported in this study, which is higher than suggested by some other estimates.

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

  https://www.mdpi.com/2072-6694/12/10/2921

  Future studies on CTCL epidemiology should use a prospective design to include data on survival. Information is also needed on the staging of lymphomas, including the non-MF/SS types. The inclusion of treatments and outcomes in such registries would help achieve progress in patient care. An independent central review of the included cases could ensure the use of the same classification system and improve the precision of the diagnosis.

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

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7600606/

  Information on the incidence of CTCL could be derived from some of the studies. Based on selected states, in the USA the incidence was estimated to be 0.770.87/100,000 person-years, whereas others reported an incidence of 0.64/100,000 person-years. In Europe, the incidence was estimated to be 0.290.39/100,000 person-years, with a possible increase over time. […] Future studies on CTCL epidemiology should use a prospective design to include data on survival. Information is also needed on the staging of lymphomas, including the non-MF/SS types. The inclusion of treatments and outcomes in such registries would help achieve progress in patient care. An independent central review of the included cases could ensure the use of the same classification system and improve the precision of the diagnosis.

• #54

  https://journals.lww.com/hemasphere/fulltext/2023/08003/p1109__epidemiology_of_cutaneous_t_cell_lymphomas_.1007.aspx

  More than 80% had an early-stage disease (IA, IB and IIA) at diagnosis and median time from first skin lesion to diagnosis, was 21 months (range, 0-562.9), higher in early phase disease than advanced stage (22.1 months vs 7.8 months, respectively; pV=0.04). […] Stage resulted as best predictor of survival. […] The real epidemiology of CTCL is still a matter of debate. […] Currently, the Italian Cutaneous Lymphoma Study-Group (ICLSG-named Commissione Linfomi Cutanei) effort is to create a national register for CTCL, based on MDT registry, to provide information from a national perspective.

• #55

  https://link.springer.com/article/10.1007/s13671-012-0038-2

  MF and SS appears to be slightly increasing in incidence over the past 10 years and while the precise triggers remain unclear, improved immunohistochemistry, immunologic and molecular biological techniques and updated clinical staging recommendations have enabled us to better characterize different subsets of patients in terms of accurate staging, genetic expression, clinical presentation and behavior, and overall prognosis.

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