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

Publication

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

Journal Paper/Review - Oct 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

Citation

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.

Type

Journal Paper/Review (English)

Journal

Acta Derm Venereol 2018; 98

Publication Date

Oct 10, 2018

Issn Electronic

1651-2057

Pages

835-841

Brief description/objective

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.