Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study.
Marie-Charlotte Brüggen,Katrin Kerl,Eugenia Haralambieva,Urs Schanz,Yun-Tsan Chang,Desislava Ignatova,Reinhard Dummer,Antonio Cozzio,Wolfram Hoetzenecker,Lars E. French,Emmanuella Guenova,Emmanuella Guenova +11 more
TL;DR: This retrospective, descriptive, monocentric, cross-sectional case study, identified 4 rare aggressive T-/NK-cell lymphomas with manifestation in the skin, which were diagnosed in a tertiary care centre over a period of 4 years.
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Abstract: Rare T- or NK-cell lymphomas with cutaneous manifestation may display a highly aggressive clinical course and major diagnostic/therapeutic challenges. This report describes our experiences with different lymphomas of this rare category and the therapeutic options used. This retrospective, descriptive, monocentric, cross-sectional case study, identified 4 rare aggressive T-/NK-cell lymphomas with manifestation in the skin, which were diagnosed in a tertiary care centre over a period of 4 years. Two patients had an Epstein-Barr virus-associated extranodal NK/T-cell lymphoma and 2 patients had a primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma. Concomitant extracutaneous involvement was observed in 2 of all 4 patients. Two patients had fulminant disease progression and resistance to chemotherapy. Two patients underwent allogeneic haematopoietic stem cell transplantation, which resulted in one complete remission and one partial remission. This report emphasizes the importance of an early diagnostic work-up and a prompt aggressive therapeutic approach.
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Mycosis fungoides and Sézary syndrome.
TL;DR: The treatment of mycosis fungoides (MF) and Sezary syndrome (SS) is dependent on the disease stage as discussed by the authors and the treatment approach is symptom oriented and aims to reduce the tumor burden and improve health-related quality of life.
14
Interferon alfa-2a maintenance after salvage autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement.
Miriam Doerschner,Agnes Pekar-Lukacs,Olivia Messerli-Odermatt,Corina Dommann-Scherrer,Markus F. Rütti,Antonia M.S. Müller,Gayathri Nair,Jivko Kamarachev,Katrin Kerl,Marc Beer,Michael Messerli,Katrin Frauenknecht,Eugenia Haralambieva,Wolfram Hoetzenecker,Lars E. French,Emmanuella Guenova +15 more
TL;DR: It is reported on a rare case of an atypical predominantly CD8+ folliculotropic MF, a subtype of MF with poorer prognosis, in a 59‐year‐old woman, and 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 autologously SCT.
9
Mycosis fungoides und Sézary-Syndrom.
TL;DR: In this paper, the authors discuss the relationship between mycosis fungoides (MF) and Sezary-Syndrom (SS) in terms of their treatment.
4
Linfoma primario cutáneo de células T epidermotropo agresivo CD8
María Belén Mariucci Vázquez,Mariana Arias,Alejandra Abeldaño +2 more
- 29 Mar 2021
TL;DR: Si bien esta entidad es un reto terapéutico, el tratamiento inicial se basa en poliquimioterapia y/o trasplante de células hematopoyéticas, con una sobrevida a los 5 años menor del 40%.
Case 36. Primary cutaneous aggressive epidermotropic T-cell lymphoma as a composite lymphoma with B-cell chronic lymphocytic leukemia
Florentia Dimitriou,Marie-Charlotte Brüggen,Emmanuella Guenova +2 more
- 01 Jan 2021
TL;DR: The CD8 marker is not unique to primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma (pc CD8+, AECTCL) as mentioned in this paper.
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