S2k Guidelines – Cutaneous Lymphomas Update 2016 – Part 2: Treatment and Follow‐up (ICD10 C82 ‐ C86)

Dippel, Edgar; Assaf, Chalid; Becker, Jürgen C.; Bergwelt‐Baildon, Michael von; Beyer, Marc; Cozzio, Antonio; Eich, Hans Theodor; Follmann, Markus; Grabbe, Stephan; Hillen, Uwe; Klapper, Wolfram; Klemke, Claus‐Detlev; Lamos, Cristina; Loquai, Carmen; Meiß, Frank; Mestel, Dominik; Nashan, Dorothée; Nicolay, Jan P.; Oschlies, Ilske; Schlaak, Max; Stoll, Christoph; Vag, Tibor; Weichenthal, Michael; Wobser, Marion; Stadler, Rudolf

doi:10.1111/ddg.13401

Cited by 40 publications

(58 citation statements)

References 71 publications

(64 reference statements)

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“…13 The management of advanced MF is currently based on monotherapy or combination therapy, including IFN-a, retinoids (bexarotene), photopheresis, chemotherapy, radiotherapy and targeted therapies such as the monoclonal antibody brentuximab. [35][36][37] Most treatment options only induce remissions of limited duration. [29][30][31] An additional challenge in the management of patients with advanced MF is their severely compromised immunity, with an aberrant T helper 2 phenotype and abnormal natural killer cell, cytotoxic T-cell and dendritic cell function.…”

Section: Discussionmentioning

confidence: 99%

Interferon alfa‐2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement

Doerschner

Pekar-Lukacs²,

Messerli-Odermatt

et al. 2019

Br J Dermatol

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Summary Mycosis fungoides (MF) is a primary cutaneous T‐cell lymphoma with unfavourable prognosis for patients with advanced stages of the disease. Refractory disease and advanced‐stage disease require systemic therapy. We report on a rare case of an atypical predominantly CD8+ folliculotropic MF, a subtype of MF with poorer prognosis, in a 59‐year‐old woman. She was initially diagnosed with MF restricted to the skin, of T3N0M0B0/stage IIB according to the current World Health Organization–European Organisation for Research and Treatment of Cancer classification. First‐line treatment with local percutaneous radiotherapy in combination with systemic interferon alfa‐2a resulted in complete remission. However, 21 months later the disease progressed to T3N0M1B0/stage IVB with development of cerebral manifestation and thus very poor prognosis. Allogeneic stem cell transplantation (SCT) was not a therapeutic option due to the lack of a suitable donor. We initiated methotrexate and cytarabine chemotherapy, followed by high‐dose chemotherapy with thiotepa and carmustine with autologous SCT. Despite rapid response and complete remission of the cerebral lesions, disease recurrence of the skin occurred soon after. Interestingly, readministration of interferon alfa‐2a as a maintenance treatment after the salvage autologous SCT resulted in a durable complete remission during the follow‐up period of currently 17 months after autologous SCT. What's already known about this topic? Mycosis fungoides is a primary cutaneous T‐cell lymphoma with unfavourable prognosis for the advanced stages of the disease. A refractory course of disease requires systemic therapy. What does this study add? We report on an unusual case of a patient with mycosis fungoides with cerebral involvement, in which a durable complete remission was achieved upon autologous stem cell therapy and interferon alfa‐2a maintenance therapy.

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Section: Discussionmentioning

confidence: 99%

Interferon alfa‐2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement

Doerschner

Pekar-Lukacs²,

Messerli-Odermatt

et al. 2019

Br J Dermatol

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“…Appropriate staging examinations included full blood count (Xn, Sysmex, Norderstedt, Germany), lactate dehydrogenase, turbidimetric analysis for direct detection and quantification of monoclonal immunoglobulins (Cobas Systems, Roche Diagnostics, Mannheim, Germany), serum protein electrophoresis (Hydrasys, Sebia, Evry, France), imaging examinations (ultrasound of lymph nodes and abdomen or computed tomography) and, in selected cases, bone marrow biopsy to exclude any systemic lymphoma manifestations. Tumour stage and the appropriate therapy were established according to international recommendations (23) and the German guidelines for cutaneous lymphomas (24,25). Follow-up investigations included regular laboratory tests (blood and urine) with a varying panel of parameters.…”

Section: Case Selection and Data Assessmentmentioning

confidence: 99%

Paraproteinaemia in Primary Cutaneous Marginal Zone Lymphoma

Frings

Röding²,

Schmeißer³

et al. 2018

Acta Derm Venerol

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Primary cutaneous marginal zone lymphomas (PCMZL) frequently exhibit lymphoplasmacytoid/plasmacytic differentiation, implying the capacity to produce monoclonal immunoglobulins. As these paraproteins are secreted, and thus are measurable in blood and urine, they may correlate with disease burden and serve as tumour markers reflecting therapeutic response. This study retrospectively analysed the records of 23 patients with PCMZL. During treatment and follow-up, laboratory tests, including full blood count, lactate dehydrogenase, serum protein electrophoresis and turbidimetric analyses, were conducted. Thirty-nine percent of cases showed a suspicious serum protein electrophoresis in terms of paraproteinaemia. In 44% of cases the heavy and light chain restriction in tissue samples correlated with serological findings. Altogether, 89% of the PCMZL patients with paraproteinaemia eventually experienced a relapse, in contrast to 62% of the group without paraproteinaemia. This study analysed the incidence and clinical implications of paraproteinaemia in patients with PCMZL. A clear correlation was found between paraproteinaemia, tumour relapse and therapeutic intervention.

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“…Primäres Ziel ist daher die Erkrankungskontrolle mit Überführung in eine lang anhaltende Remission unter Erhaltung der Lebensqualität. Das therapeutische Ansprechen variiert hierbei in Abhängigkeit vom Stadium der Erkrankung, den Vortherapien, der Expression bestimmter Zielantigene auf der Tumoroberfläche und vermutlich auch der Zusammensetzung des Tumormikromilieus (tumor microenvironment, TMEs) . Neben neuen Antikörpertherapien, wie dem CCR4‐Antikörper Mogamulizumab und dem CD30‐Antikörper Brentuximab Vedotin, existieren auch epigenetisch wirksame Medikamente (Histon Deacetylase (HDAC)‐Inhibitoren), die jedoch in Europa bisher nicht zugelassen sind .…”

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Kutane Lymphome – neue Perspektiven durch Kombinationstherapien?

Mitteldorf

2020

J Deutsche Derma Gesell

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S2k Guidelines – Cutaneous Lymphomas Update 2016 – Part 2: Treatment and Follow‐up (ICD10 C82 ‐ C86)

Cited by 40 publications

References 71 publications

Interferon alfa‐2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement

Interferon alfa‐2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement

Paraproteinaemia in Primary Cutaneous Marginal Zone Lymphoma

Kutane Lymphome – neue Perspektiven durch Kombinationstherapien?

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