Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study
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, descriptiv...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Medical Journals Sweden
2018-07-01
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| Series: | Acta Dermato-Venereologica |
| Subjects: | |
| Online Access: |
https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2950
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| _version_ | 1798031212729597952 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Marie-Charlotte Brüggen |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-11T19:53:47Z |
| format | Article |
| id | doaj.art-70fbda1f6fe84108b577265160ad9f1c |
| institution | Directory Open Access Journal |
| issn | 0001-5555 1651-2057 |
| language | English |
| last_indexed | 2024-04-11T19:53:47Z |
| publishDate | 2018-07-01 |
| publisher | Medical Journals Sweden |
| record_format | Article |
| series | Acta Dermato-Venereologica |
| spelling | doaj.art-70fbda1f6fe84108b577265160ad9f1c2022-12-22T04:06:14ZengMedical Journals SwedenActa Dermato-Venereologica0001-55551651-20572018-07-0198983584110.2340/00015555-29505221Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case StudyMarie-Charlotte Brüggen0Katrin KerlEugenia HaralambievaUrs SchanzYun-Tsan ChangDesislava IgnatovaReinhard DummerAntonio CozzioWolfram HoetzeneckerLars E. FrenchEmmanuella Guenova Department of Dermatology, University Hospital Zurich, CH-8091 Zurich, Switzerland. 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. https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2950 lymphomamanifestationintheskinENKTLPC-AECTLallogeneichematopoieticstemcelltransplantation |
| spellingShingle | 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 Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study Acta Dermato-Venereologica lymphoma manifestationintheskin ENKTL PC-AECTL allogeneichematopoieticstemcelltransplantation |
| title | Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study |
| title_full | Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study |
| title_fullStr | Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study |
| title_full_unstemmed | Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study |
| title_short | Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study |
| title_sort | aggressive rare t cell lymphomas with manifestation in the skin a monocentric cross sectional case study |
| topic | lymphoma manifestationintheskin ENKTL PC-AECTL allogeneichematopoieticstemcelltransplantation |
| url |
https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2950
|
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