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

Publikation

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

Wissenschaftlicher Artikel/Review - 10.10.2018

Brüggen Marie-Charlotte, French Lars E, Hoetzenecker Wolfram, Cozzio Antonio, Dummer Reinhard, Desislava Ignatova, Chang Yun-Tsan, Schanz Urs, Haralambieva Eugenia, Kerl Katrin, Guenova Emmanuella

Zitation

Brüggen M, French L, Hoetzenecker W, Cozzio A, Dummer R, Desislava I, Chang Y, Schanz U, Haralambieva E, Kerl K, Guenova E. Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study. Acta Derm Venereol 2018; 98:835-841.

Art

Wissenschaftlicher Artikel/Review (Englisch)

Zeitschrift

Acta Derm Venereol 2018; 98

Veröffentlichungsdatum

10.10.2018

eISSN (Online)

1651-2057

Seiten

835-841

Kurzbeschreibung/Zielsetzung

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.