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Mycosis fungoides and Sézary syndrome are rare and largely incurable types of cutaneous T-cell lymphoma with limited therapeutic options. In 1984 Bunn et al. reported that interferon alpha is an efficient monotherapy in cutaneous T-cell lymphoma and 14 years later it was shown in a prospective, randomized trial that a combination of interferon alpha and psoralen plus ultraviolet A therapy (PUVA) is most efficient in the treatment of cutaneous T-cell lymphoma. Since then interferon alpha as single agent or, most often, in combination with phototherapy and/or retinoids has been integrated as standard of care in cutaneous T-cell lymphoma guidelines worldwide. However, production of interferon alpha was discontinued recently worldwide and pegylated interferon alpha-2a (PEG-IFNα) has been used as an alternative therapy. In contrast to numerous interferon alpha studies, only a few studies focusing on PEG-IFNα are available. Therefore, the aim of this study was to conduct a retrospective data collection to report on the efficacy, adverse events and therapy regimens of PEG-IFNα in cutaneous T-cell lymphoma. In 28 patients with cutaneous T-cell lymphoma treated in Germany and in the Netherlands, 36% of patients achieved complete remission, 36% partial remission and 29% stable disease. Eighteen percent of patients developed adverse events during therapy, which led to the discontinuation of PEG-IFNα therapy in 2 patients. The most common concomittant therapies were oral PUVA phototherapy and local radiotherapy. In conclusion, PEG-IFNα, especially in combination with skin-directed therapies, is an effective treatment option for cutaneous T-cell lymphoma in clinical practice. [ABSTRACT FROM AUTHOR]

Background: Interferon-alpha is an important therapeutic option for the treatment of the cutaneous T-cell lymphomas (CTCL). Since the approved recombinant interferon-α-2a (IFN-α2a) has no longer been produced since January 2020, pegylated interferon-α2a (pegIFN-α2a) can be used as an alternative treatment, even though it is not approved for the treatment of CTCL. The aim of this multicentre study was to generate comprehensive data on the efficacy and tolerability of pegIFN-α2a in the treatment of CTCL., Patients and Methods: A multicenter retrospective study was conducted with 70 patients with CTCL from twelve German skin centers., Results: In total, 70 patients were included in the study, with 57.2% male and a mean age of 58.8 ± 14.9 years. Mycosis fungoides was present in 71.4% of cases and Sézary Syndrome in 28.6%. An overall response rate of 55.2% was observed with pegIFNα-2a therapy. In 50% of cases, therapy was discontinued after 63.6 ± 33.5 weeks. The most common reason for discontinuation was adverse events, which occurred in 68.6% of cases and which were classified as severe in 29.2%. Blood count changes, fatigue and liver toxicity occurred most frequently., Conclusions: Our analysis provides comprehensive data on the efficacy and tolerability of pegIFNα-2a therapy in patients with CTCL. In terms of response rates and side effect profile, pegIFNα-2a appears to be comparable to IFN-α2a therapy., (© 2024 The Author(s). Journal der Deutschen Dermatologischen Gesellschaft published by Wiley‐VCH GmbH on behalf of Deutsche Dermatologische Gesellschaft.)

Background: Primary cutaneous lymphomas (PCL) are rare skin tumors of lymphoproliferative neoplasms and belong to the heterogeneous group of non-Hodgkin's lymphomas. PCL encompass a broad spectrum of clinical and histologic manifestations, with cutaneous T-cell lymphoma (CTCL) being the most common (73%). Due to the rarity of the diseases, population-based studies of care and epidemiology are limited., Patients and Methods: Based on anonymized, age- and sex-adjusted SHI (statutory health insurance) claims data of approximately five million SHI-insured patients, a retrospective analysis was conducted over a six-year period (2012-2017) to determine the prevalence, incidence, and lethality in patients with mature-cell T/NK-cell lymphoma in Germany., Results: A total of 1,336 patients with T-cell lymphoma were identified during the observation period. The six-year prevalence ranged from 27.35 to 43.58 per 100,000. Patients were 65% male with a mean age of 66 years (SD 15). There were 246 patients (approx. 20%) who died within the 6 years, up to 7% per year. The calculated incidence in 153 identified patients in 2017 is 3.65 to 3.92 per 100,000., Conclusions: For the first time, valid epidemiologic findings of patients with mature T-cell and NK-cell lymphomas were obtained using SHI claims data in Germany. Further analyses are needed to gain a deeper insight into the healthcare reality of patients with this rare disease., (© 2023 Kyowa Kirin Gmbh and The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by John Wiley & Sons Ltd.)

Primary cutaneous lymphoma (CL) form a heterogenous group of non-Hodgkin lymphoma (NHL). The CL are divided into T cell lymphoma (CTCL), these form the majority of CL with 73%, followed by B cell lymphoma (CBCL) with 22% and the rare lymphoma build 5% based on the current WHO-classification. There are only few studies, which treated the CL topic and its incidence, prevalence and survival in Germany. The last study has been published in the year 2007 including 998 patients from 26 different centers. However, recent studies from other national registries show a slight increasing tendency of incidence and prevalence of CL. Therefore, the underlying study investigated aged-standardized incidence rates and 5-year survival of CL for the time period 2008–2017 with data of the population-based cancer registry (LKR) of North Rhine-Westphalia, the largest Federal State of Germany (18 million inhabitants). Overall, 2826 patients with CL were registered in the period of 2008 till 2017 of the cancer registry. The LKR is a reporting point of all forms of cancer, based on an obligated report for all pathologists and dermatopathologists since 2005. The data is allocated as followed 1743 male (61.7%) and 1083 female (38.3%) with a rate of male>female (1.6:1). The present work shows based on the registration of 2826 patients the dominance of cutaneous T-cell-lymphoma (68%) in comparison to cutaneous B-cell-Lymphoma (24.9%), however we also divided the primary cutaneous B cell lymphoma (CBCL) in one overall category (n=905) and three subtypes (n=705): follicular B cell lymphoma (C82), diffuse large B cell lymphoma (C83.3) and marginal zone lymphoma; C88.40), with the following distribution 37.7%, 30.2%, 32.1%. The age-standardized incidence rates (per 100 000 person-years) of cutaneous lymphoma were 1,5 and 0,8 among men and women. Five-year absolute survival was 79.1% in MF, and 77.3% in all CBCL and 88.2% in marginal zone lymphoma, 89.6% in follicular lymphoma and 49.6% in diffuse large B cell lymphoma, respectively. Overall, there is a stable incidence in CTCL and a slightly increasing incidence of CBCL (0.14 in 2008 to 0.34 in 2017). This progression in CBCL may be due to higher awareness and clear substratification based on the recent classifications. [ABSTRACT FROM AUTHOR]

Background: In addition to a broad and clinically diverse spectrum of known primary cutaneous lymphomas, for which an incidence of 1-3:100,000 is postulated, each year further entities are specified and defined. The goal is the presentation of a case series from daily clinical routine., Methods: Over a period of 6 years and 2 months, patients consulting the Department of Dermatology, Medical Center University of Freiburg, were registered. Subsequently, collectives of mycosis fungoides (MF), Sezary syndrome (SS), CD30+ lymphoproliferative diseases, single cases with rare primary cutaneous lymphomas, and subcollectives of B‑cell lymphomas were examined. The high number of MF cases allowed the additional quantitative analyses of the types of therapies used in this group., Results: Yearly 16-25 new diagnoses of primary cutaneous lymphoma are made. The evaluation of 163 primary cutaneous lymphoma revealed 111 cases with MF (68.1%), including 9 particular variants, 15 primary cutaneous CD30+ lymphoproliferative diseases (9.2%) dominated by 10 lymphomatoid papulosis (LyP), in addition to 5 primary cutaneous anaplastic large cell lymphoma (PCALCL), 6 SS (3.68%), and 24 cutaneous B‑cell lymphomas (14-72%). Three cases with rare primary cutaneous T/NK cell lymphomas are addressed in detail. In all, 82% of MF cases were stage IA and IB. The descending use of therapies for stage I-III included steroids and diverse UV therapies followed by bexarotene, interferon-α, methotrexate, and extracorporal photophoresis., Conclusions: Diagnoses of cutaneous lymphomas belong to a vast spectrum of differential diagnoses. This registry describes frequent findings and shows rare variants. You can only diagnose what you know; accordingly, a collection of case reports, which we wish to encourage, can help in processing and specification of entities.