Challenges in early diagnosis of primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma: a case seri
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Clinical Report Nahide ONSUN1 Didem DIZMAN1 1 ˘ Nazan EMIROGLU Pelin YILDIZ2 Tahsin C ¸ a˘gdas¸ AKASLAN1 Zeynep TOSUNER3 Güven C ¸ ETIN4 Cuyan DEMIRKESEN3 1
Department of Dermatology, Bezmialem Vakif University Faculty of Medicine, 2 Department of Pathology, Bezmialem Vakif University Faculty of Medicine, 3 Acibadem University Faculty of Medicine, Department of Pathology, 4 Department of Haematology, Bezmialem Vakif University Faculty of Medicine, ˙Istanbul Turkey
Reprints: Nahide Onsun
Article accepted on 03/04/2020
Challenges in early diagnosis of primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma: a case series of four patients Background: Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma (AECTCL) is a rare and aggressive lymphoma characterised by ulcerated lesions and a poor prognosis. Objectives: To present a case series of four previously misdiagnosed AECTCL patients and discuss the importance of early diagnosis. Materials and Methods: All patients in this study were identified from the database of the Dermatology Department of the Medical School of Bezmialem Vakif University, based on clinical and histopathological diagnosis of AECTCL between 2010 and 2018. Results: AECTCL cases may mimic many benign dermatoses and accurate diagnosis may be delayed. Conclusion: Because of its poor prognosis, early diagnosis of AECTCL may be helpful in improving the likelihood of patient survival, but further study is needed to address the challenges in diagnosing this rare and aggressive lymphoma Key words: aggressive, epidermotropic, CD8 + , cutaneous T-cell lymphoma
P
rimary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma is a rare and poorly characterized variant of cutaneous lymphoma. The World Health Organisation (WHO) and the European Organisation for Research and Treatment (EORTC) classify primary cutaneous lymphomas based on a combination of clinical, histological, immunohistochemical, and genetic criteria. Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma (AECTCL) was included as a provisional entity in the WHO-EORTC classification for primary cutaneous lymphomas in 2005 as well as in the 2018 update [1,2].
Case 1
Case 2
A 46-year-old man presented with a two-year history of ulcerated vegetative mass on the right thumb and viola-
A 70-year-old man presented with ulcerated lesions on his trunk. He had been diagnosed with lymphomatoid
Methods
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EJD, vol. 30, n◦ 4, July-August 2020
To cite this article: Onsun N, Dizman D, Emiro˘glu N, Yildiz P, Akaslan TC¸, Tosuner Z, C¸etin G, Demirkesen C. Challenges in early diagnosis of primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma: a case series of four patients. Eur J Dermatol 2020; 30(4): 358-61 doi:10.1684/ejd.2020.3843
doi:10.1684/ejd.2020.3843
All patients in this study were identified from the database of the Dermatology Department of the Medical School of Bezmialem Vakif University, based on clinical and histopathological diagnosis of A
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