Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study.

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.
Authors	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
Journal	Acta dermato-venereologica (Acta Derm Venereol) Vol. 98 Issue 9 Pg. 835-841 (Oct 10 2018) ISSN: 1651-2057 [Electronic] Sweden
PMID	29693696 (Publication Type: Case Reports, Journal Article)
Chemical References	Antineoplastic Agents
Topics	Aged Aged, 80 and over Antineoplastic Agents (therapeutic use) Cross-Sectional Studies Disease Progression Drug Resistance, Neoplasm Early Detection of Cancer Fatal Outcome Female Hematopoietic Stem Cell Transplantation Herpesvirus 4, Human (isolation & purification) Humans Lymphoma, Extranodal NK-T-Cell (immunology, pathology, therapy, virology) Lymphoma, T-Cell, Cutaneous (immunology, pathology, therapy) Male Middle Aged Retrospective Studies Skin Neoplasms (immunology, pathology, therapy, virology) Switzerland T-Lymphocytes, Cytotoxic (immunology, pathology) Time Factors Treatment Outcome

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