In:
Histopathology, Wiley, Vol. 67, No. 4 ( 2015-10), p. 425-441
Kurzfassung:
Aggressive epidermotropic cutaneous CD 8 + lymphoma is currently afforded provisional status in the WHO classification of lymphomas. An EORTC Workshop was convened to describe in detail the features of this putative neoplasm and evaluate its nosological status with respect to other cutaneous CD 8 + lymphomas. Methods and results Sixty‐one CD 8 + cases were analysed at the workshop; clinical details, often with photographs, histological sections, immunohistochemical results, treatment and patient outcome were discussed and recorded. Eighteen cases had distinct features and conformed to the diagnosis of aggressive epidermotropic cutaneous CD 8 + lymphoma. The patients typically present with widespread plaques and tumours, often ulcerated and haemorrhagic, and histologically have striking pagetoid epidermotrophism. A CD 8 + / CD 45 RA + / CD 45 RO − / CD 2 − / CD 5 − / CD 56 − phenotype, with one or more cytotoxic markers, was found in seven of 18 patients, with a very similar phenotype in the remainder. The tumours seldom involve lymph nodes, but mucosal and central nervous system involvement are not uncommon. The prognosis is poor, with a median survival of 12 months. Examples of CD 8 + mycosis fungoides, lymphomatoid papulosis and Woringer–Kolopp disease presented the typical features well documented in the CD 4 + forms of those diseases. Conclusions Aggressive epidermotropic cutaneous CD 8 + lymphoma is a distinct lymphoma that warrants inclusion as a distinct entity in future revisions of lymphoma classifications.
Materialart:
Online-Ressource
ISSN:
0309-0167
,
1365-2559
DOI:
10.1111/his.2015.67.issue-4
Sprache:
Englisch
Verlag:
Wiley
Publikationsdatum:
2015
ZDB Id:
2006447-0
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