Materiały źródłowe

• #1 JMIR Research Protocols – Predictive and Prognostic Biomarkers in Patients With Mycosis Fungoides and Sézary Syndrome (BIO-MUSE): Protocol for a Translational Study

  https://www.researchprotocols.org/2024/1/e55723

  Cutaneous T-cell lymphoma (CTCL) is a rare group of lymphomas that primarily affects the skin. Mycosis fungoides (MF) is the most common form of CTCL and Szary syndrome (SS) is more infrequent. Early stages (IA-IIA) have a favorable prognosis, while advanced stages (IIB-IVB) have a worse prognosis. Around 25% of patients with early stages of the disease will progress to advanced stages. […] The TNMB stage at diagnosis remains the most important prognostic factor. Many patients with early stages of MF have an indolent disease with a 5-year disease-specific survival of 89% to 98%. However, approximately 25% of patients with early stages of disease will later progress to advanced stages. Patients with advanced stages of MF have a 5-year disease-specific survival of 18% to 56% associated with treatment failure. The 5-year disease-specific survival of SS is 36%.

• #2 JMIR Research Protocols – Predictive and Prognostic Biomarkers in Patients With Mycosis Fungoides and Sézary Syndrome (BIO-MUSE): Protocol for a Translational Study

  https://www.researchprotocols.org/2024/1/e55723/

  Cutaneous T-cell lymphoma (CTCL) is a rare group of lymphomas that primarily affects the skin. Mycosis fungoides (MF) is the most common form of CTCL and Szary syndrome (SS) is more infrequent. Early stages (IA-IIA) have a favorable prognosis, while advanced stages (IIB-IVB) have a worse prognosis. Around 25% of patients with early stages of the disease will progress to advanced stages. Patients with advanced stages of MF have a 5-year disease-specific survival of 18% to 56% associated with treatment failure. The 5-year disease-specific survival of SS is 36%. […] The TNMB stage at diagnosis remains the most important prognostic factor. Many patients with early stages of MF have an indolent disease with a 5-year disease-specific survival of 89% to 98%. However, approximately 25% of patients with early stages of disease will later progress to advanced stages.

• #3 How Long Will I Live With CTCL? – HealthTree for Cutaneous T-Cell Lymphoma

  https://healthtree.org/cutaneous-t-cell-lymphoma/community/how-long-will-i-live-with-cutaneous-t-cell-lymphoma

  The prognosis for cutaneous T-cell lymphoma (CTCL) varies greatly depending on several factors, including the stage of the disease at diagnosis, the patient’s overall health, and the specific subtype of CTCL. It’s important to note that while statistics can provide a general idea, they cannot predict the exact outcome for an individual patient. […] Early-stage mycosis fungoides has been associated with a long survival. A recent study from 2019 looking at stage IBIVB mycosis fungoides (MF) and Sezary syndrome found that 10-20% of stage IB patients don’t survive 5 years while patients with advanced-stage mycosis fungoides and Sezary syndrome have a 5-year survival of about 20-60%. […] Another study found that for those with mycosis fungoides, the predicted 10-year overall survival rate was 90.3% for early-stage disease compared to 53.2% for advanced-stage disease.

• #4 Cutaneous T-Cell Lymphoma (CTCL)

  https://my.clevelandclinic.org/health/diseases/17940-cutaneous-t-cell-lymphoma

  Most CTCLs grow very slowly and aren’t life-threatening. […] Healthcare providers can’t cure CTCLs, but they can successfully manage symptoms with treatment. […] But like all cancers, CTCLs can be fatal in their advanced stages. The sooner you start treatment, the better. […] The overall 10-year survival rate for people with early-stage (Stage I or II) CTCL is 90%. That means that 9 out of 10 people with this condition are still alive 10 years later. People with late-stage CTCL (Stage III or IV) have an overall 10-year survival rate of 53%. […] Survival rates are estimates. They can’t tell you how long you’ll live or how you’ll respond to treatment. To learn more about survival rates and what they mean for you, ask your healthcare provider.

• #5 Cutaneous T-cell Lymphoma | Cutaneous Lymphoma Foundation

  https://www.clfoundation.org/cutaneous-t-cell-lymphoma

  There is no known cure for CTCL, though some patients have long-term remission with treatment and many more live symptom-free for many, many years. […] Research indicates that most patients diagnosed with CTCL (mycosis fungoides type) have early stage disease, and have a normal life expectancy. […] With advances in research and new treatment options resulting from physician collaboration and clinical trials, CTCL patients are experiencing better care and an array of effective treatment options that work for them.

• #6 Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq

  The prognosis of patients with cutaneous T-cell lymphomas is based on the extent of disease (stage) at presentation. The presence of lymphadenopathy and involvement of peripheral blood and viscera increase in likelihood with worsening cutaneous involvement and define poor prognostic groups. The Cutaneous Lymphoma International Consortium retrospectively reviewed 1,275 patients and found that the following four independent prognostic markers indicate a worse survival: Stage IV disease, Age older than 60 years, Large cell transformation, Elevated lactate dehydrogenase. The median survival following diagnosis varies according to stage. Patients with stage IA disease have a median survival of 20 years or more. Most deaths for this group are not caused by, nor are they related to, mycosis fungoides. In contrast, more than 50% of patients with stage III through stage IV disease die of mycosis fungoides, with a median survival of approximately 5 years. The Cutaneous Lymphoma International Prognostic index used male sex, age older than 60 years, plaques, lymph nodes, blood involvement, and visceral involvement as poor prognostic factors to define predicted overall survival (OS) and progression-free survival in both early-stage and advanced-stage groups. There is consensus that patients with Szary syndrome (leukemic involvement) have a poor prognosis (median survival, 4 years), with or without the typical generalized erythroderma. Cytologic transformation from a low-grade lymphoma to a high-grade lymphoma (large cell transformation) occurs rarely (5%) during the course of these diseases and is associated with a poor prognosis. A retrospective analysis of 100 cases with large cell transformation found reduced disease-specific survival with extracutaneous transformation, increased extent of skin lesions, and CD30 negativity. A common cause of death during the tumor phase is septicemia caused by chronic skin infection with staph species, herpes simplex, herpes zoster, and fungal skin infections.

• #7 Prognosis in cutaneous T-cell lymphoma by skin stage: long-term survival in 489 patients – PubMed

  https://pubmed.ncbi.nlm.nih.gov/10071312/

  Although a number of studies have documented the long-term survival of patients with cutaneous T-cell lymphoma (CTCL), none have provided data as to the relative survival of all 4 skin stages. […] We document survival of CTCL patients by T stage relative to that of an age-, sex-, and race-matched population. […] For stage T1 ( 10% skin involved) there was no significant difference between the observed and expected survivals. For the other 3 stages the observed survival was significantly inferior to that of the expected survival (P = .002). At 10 years the relative survivals were: T2 (10% or more skin involved) 67.4%, T3 (tumor stage) 39.2%, T4 (generalized erythroderma) 41.0%. […] Lymphadenopathy had an unfavorable impact on prognosis. […] The relative survival of CTCL patients worsens with increasing skin stage, although stages T3 and T4 had closely similar survivals. The great majority of patients with CTCL do not die of their disease.

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

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

  Mycosis fungoides and Szary syndrome are incurable conditions in most patients, with the exception of those with stage IA disease. […] Mortality and prognosis are related to the disease stage at diagnosis, as well as to the type and extent of skin lesions and whether or not extracutaneous disease is present, as demonstrated by the following: Patients diagnosed with stage IA mycosis fungoides (patch or plaque skin disease limited to 10% of the skin surface area) who undergo treatment have an overall life expectancy similar to age-, sex-, and race-matched controls (10-year survival rate of 97-98%). […] Patients who have stage IIB disease with cutaneous tumors have a median survival rate of 3.2 years (10-year survival rate of 42%). […] Patients who have stage III disease (generalized erythroderma) have a median survival rate of 4-6 years (10-year survival rate of 83%).

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

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

  Patients with extracutaneous disease stage IVA (lymph nodes) or stage IVB (viscera) have a survival rate of less than 1.5 years (10-year survival rate of 20% for patients with histologically documented lymph node involvement). […] Patients with effaced lymph nodes, visceral involvement, and transformation to large T-cell lymphoma have an aggressive clinical course and usually die of systemic involvement or infections. […] Blood eosinophilia at baseline may also serve as a prognostic factor in patients with primary cutaneous T-cell lymphoma. […] Univariate analysis has shown that the following factors are also associated with reduced survival and increased risk of disease progression: Increased age, Male sex, Increased lactate dehydrogenase (LDH). […] An analysis of 20-year trends reporting on incidence and associated mortality of patients with mycosis fungoides showed a decline in the mortality rate in the United States, perhaps related to increased recognition and earlier diagnosis of the disease.

• #10 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 (CTCL), a group of non-Hodgkin T-cell lymphomas, remains a rare disease, incidence has increased steadily since the 1970s. […] Over that same period, investigators also observed a trend toward increasing 5-year overall survival (OS) since 1973, plateauing at 78.3% from 1997 to 2005. […] The predicted 10-year overall survival (OS) rate is 90.3% for early-stage disease vs 53.2% for advanced stage disease. […] In results published in 2015, the reported median OS for patients with stage IIB disease was 68 months compared with 48 months for those with stage IVA disease and 33 months for those with stage IVB disease. 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.

• #11 JMIR Research Protocols – Predictive and Prognostic Biomarkers in Patients With Mycosis Fungoides and Sézary Syndrome (BIO-MUSE): Protocol for a Translational Study

  https://www.researchprotocols.org/2024/1/e55723/

  Multivariate analyses of cohorts of patients with MF and SS have identified potential adverse prognostic factors in the early stages of disease consisting of male gender, aged 60 years, plaques, folliculotropic disease, and stage N1/NX and negative prognostic factors for advanced disease consisting of male gender, aged 60 years, stages B1/B2 and N2/N3, and visceral involvement. […] Still, there is an unmet clinical need to identify which patients will progress to an advanced stage of disease, and there is a great need for new reliable prognostic markers. […] This study aims to find potential predictive and prognostic biomarkers in patients with MF and SS. This study will investigate translational samples from skin and blood concerning the stage of the disease over time, which has the advantage of monitoring patients with consecutive samplings to be able to capture changes before and during a possible progression. […] There is a clinically unmet need to find new predictive and prognostic biomarkers in MF and SS, and studies further exploring these areas are therefore a high priority.

• #12 Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq

  […] […] A report on 1,798 patients from the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program database found an increase in second malignancies in patients with mycosis fungoides (standardized incidence ratio, 1.32; 95% confidence interval [CI], 1.151.52), especially for Hodgkin lymphoma, non-Hodgkin lymphoma, and myeloma. Another report on 4,459 patients from the SEER database found that the 19.2% of African American patients with mycosis fungoides had shorter OS, potentially attributable to disease characteristics, socioeconomic status, and type of therapy (hazard ratio, 1.47; 95% CI, 1.251.74; P .001). […] […] […] Several retrospective studies showed that 20% of patients have disease that progresses from stage I or II to stage III or IV. Szary syndrome presents with generalized erythroderma (T4) and peripheral blood involvement. However, there is some disagreement about whether mycosis fungoides and Szary syndrome are actually variants of the same disease. The same retrospective study with a median follow-up of 14.5 years found that only 3% of 1,422 patients progressed from mycosis fungoides to Szary syndrome.

• #13 How Long Will I Live With CTCL? – HealthTree for Cutaneous T-Cell Lymphoma

  https://healthtree.org/cutaneous-t-cell-lymphoma/community/how-long-will-i-live-with-cutaneous-t-cell-lymphoma

  This 2018 study reported median overall survival for mycosis fungoides patients with stage IIB disease was 68 months compared to 48 months for those with stage IVA disease and 33 months for those with stage IVB disease. In this study, patients with the following characteristics had higher rates of disease progression: plaque lesions involving than 10% of the body surface, high lactate dehydrogenase, high beta-2-microglobulin and stage IB disease. […] The stage of CTCL at the time of diagnosis is one of the most important factors. Early-stage disease (stage I or II) generally has a better prognosis than advanced-stage disease (stage III or IV). […] Younger patients and those in good overall health typically have a better prognosis. […] There are several subtypes of CTCL, some of which have a better prognosis than others. For example, patients with mycosis fungoides generally have a better prognosis than those with Szary syndrome. […] Patients who respond well to initial treatment often have a better prognosis. […] It’s important to note that survival statistics are general trends and a patient’s individual prognosis can vary greatly. Each patient’s case is unique and should be discussed with their healthcare provider.

• #14 Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq

  […] […] Cytologic transformation from a low-grade lymphoma to a high-grade lymphoma (large cell transformation) occurs rarely (5%) during the course of these diseases and is associated with a poor prognosis. A retrospective analysis of 100 cases with large cell transformation found reduced disease-specific survival with extracutaneous transformation, increased extent of skin lesions, and CD30 negativity. […] […] […] Patients with Szary syndrome (leukemic involvement) have a poor prognosis (median survival, 4 years), with or without the typical generalized erythroderma.

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

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

  The overall mortality rate is 0.064 per 100,000 persons; however, the mortality rate widely varies depending on the stage of disease at diagnosis. […] Late-stage mycosis fungoides or Szary syndrome is associated with declining immunocompetence. […] Death most often results from systemic infection, especially with Staphylococcus aureus or Pseudomonas aeruginosa. […] Secondary malignancies, such as higher-grade non-Hodgkin lymphoma, Hodgkin disease, colon cancer, and cardiopulmonary complications (eg, high output failure, comorbid cardiopulmonary disease) also contribute to mortality. […] Median survival for patients with Szary syndrome has been reported to be 2-4 years, although the median survival was 2.9 years among patients defined by 2011 criteria for the disease. […] The disease-specific 5-year survival rate has been reported to be 24%.

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

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

  The prognosis associated with folliculotropic mycosis fungoides is worse than that for classic patch- and plaque-stage mycosis fungoides and corresponds more closely with tumor-stage disease (stage IIB). […] For this reason, some authors have proposed that the disorder be staged with tumor-stage disease (stage IIB). […] Folliculotropic mycosis fungoides reportedly has a disease-specific 5-year survival rate of approximately 70-80%. […] In patients with adult T-cell leukemia/lymphoma, the clinical subtype is the main prognostic factor. […] Survival in persons with acute or lymphomatous variants is poor, ranging from 2 weeks to more than 1 year. […] Chronic and smoldering forms have a more protracted clinical course and a longer survival period, but transformation into an acute phase with an aggressive course may occur.

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

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

  Subcutaneous panniculitis-like T-cell lymphoma (with a CD8+, alpha/beta+ T-cell phenotype) tends to have a protracted clinical course with recurrent subcutaneous nodules but without extracutaneous dissemination or the development of a hemophagocytic syndrome. […] The 5-year survival rate for such patients may be greater than 80%. […] Primary cutaneous peripheral T-cell lymphoma, unspecified (PTCL-U), is associated with a poor prognosis. […] The 5-year survival rate is less than 20%, regardless of whether the disease manifests with solitary/localized lesions or generalized skin lesions. […] Nasal-type NK/T-cell lymphoma manifesting in the skin is highly aggressive, and patients have a median survival period of less than 12 months. […] The most important factor in predicting a poor outcome is the initial presence of extracutaneous involvement.

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

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

  Patients first seen with only cutaneous involvement had a median survival of 27 months, compared with months for patients presenting with cutaneous and extracutaneous disease. […] Granulomatous cutaneous T-cell lymphomas tend to be therapy resistant, with a slowly progressive course that is apparently worse than that of classic nongranulomatous mycosis fungoides. […] Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is associated with a favorable prognosis, with an estimated 5-year disease-free survival rate of 100%. […] Patients first seen with solitary or localized skin lesions tend to have an excellent prognosis. […] Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma often has an aggressive clinical course, and patients have a median survival period of 32 months.

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

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

  Cutaneous gamma/delta-positive T-cell lymphoma patients tend to have aggressive disease resistant to multiagent chemotherapy and/or radiation, with a median survival in 1 study of only 15 months. […] A trend toward decreased survival was noted for patients with subcutaneous fat involvement, in comparison with patients who had epidermal or dermal disease only. […] Primary cutaneous acral CD8+ T-cell lymphoma carries an excellent prognosis.

• #20 JMIR Research Protocols – Predictive and Prognostic Biomarkers in Patients With Mycosis Fungoides and Sézary Syndrome (BIO-MUSE): Protocol for a Translational Study

  https://www.researchprotocols.org/2024/1/e55723

  Still, there is an unmet clinical need to identify which patients will progress to an advanced stage of disease, and there is a great need for new reliable prognostic markers. […] This study aims to find potential predictive and prognostic biomarkers in patients with MF and SS. This study will investigate translational samples from skin and blood concerning the stage of the disease over time, which has the advantage of monitoring patients with consecutive samplings to be able to capture changes before and during a possible progression. There is a clinically unmet need to find new predictive and prognostic biomarkers in MF and SS, and studies further exploring these areas are therefore a high priority.

• #21 Immune cell topography predicts response to PD-1 blockade in cutaneous T cell lymphoma | Nature Communications

  https://www.nature.com/articles/s41467-021-26974-6

  Cutaneous T cell lymphomas (CTCL) are rare but aggressive cancers without effective treatments. While a subset of patients derive benefit from PD-1 blockade, there is a critically unmet need for predictive biomarkers of response. […] Approximately one-third of CTCL patients present with advanced-stage disease, which has a 5-year survival rate of 28%. With the exception of hematopoietic stem cell transplantation, there are no effective or sustainable therapies for advanced CTCL. […] A recent Cancer Immunotherapy Trials Network (CITN) multicenter phase II clinical trial (NCT02243579) of the anti-PD-1 immunotherapy, pembrolizumab, in advanced cutaneous T cell lymphoma (CTCL) showed that 38% of patients achieved a sustained clinical response, whereas 25% experienced disease progression. […] These results highlight the importance of spatial cellular organizationnamely a distancing balance of effector and immunosuppressive T cell activityfor predicting anti-PD-1 immunotherapy response in CTCL.

• #22 Immune cell topography predicts response to PD-1 blockade in cutaneous T cell lymphoma | Nature Communications

  https://www.nature.com/articles/s41467-021-26974-6

  The expression of key T cell, macrophage and PD-1 signaling markers was assessed by standard single-plex IHC for each patient at baseline. No differences were observed for these eight markers, as shown for CD4, FoxP3, PD-1, and PD-L1. […] These findings indicate that 1) nonresponders have an immunosuppressed phenotype relative to responders, 2) only responders develop an immune-activated TME following pembrolizumab treatment, and 3) the functional immune state of the CTCL TME depends on factors beyond raw immune cell counts. […] Importantly, these spatial interaction differences (i.e., CN-5, CN-8, CN-10) between patient groups occurred even though there were no differences in the abundance of dendritic cells, CD4+ T cells, Tregs, or tumor cells. […] The SpatialScore was calculated on a per-cell basis and the mean value was reported for each patient group. When calculated with all CD4+ T cells, the pretreatment SpatialScore was significantly lower in responders than nonresponders, with enhancement post-treatment.

• #23 How to predict the outcome in mature T and NK cell lymphoma by currently used prognostic models? | Blood Cancer Journal

  https://www.nature.com/articles/bcj201223

  To select an appropriate prognostic model in the treatment of mature T- and natural killer (NK) -cell lymphoma (peripheral T-cell lymphoma (PTCL) and NK-/T-cell lymphoma (NKTCL)) is crucial. […] The correlation between complete response (CR) rate, 3-year overall survival (OS), early mortality rate and four prognostic models was analyzed. […] Three-year OS rate was 34.7%, and anaplastic large-cell lymphoma harbored better outcome than others. […] IPI score had the lowest Akaike information criterion value (1081.197) and was the best score in predicting OS and early mortality (P=0.009). […] Ann Arbor stage classification can predict CR rate more precisely (P=0.006). […] OS was significantly better in patients who received HSCT, even in patients with unfavorable features compared with chemotherapy alone. […] All prognostic models were useful to evaluate the outcome of patients with PTCL and NKTCL but IPI score did best in predicting OS in PTCL and PIT score in NKTCL. […] This study also supported the role of HSCT in patients with high-risk or refractory PTCL or NKTCL.

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