CD56-positive cutaneous lymphoma: A poorly recognized entity in the spectrum of primary cutaneous disease

P. Savoia, M. T. Fierro, M. Novelli, P. Quaglino, A. Verrone, M. Geuna, M. G. Bernengo

Research output: Contribution to journalArticlepeer-review

Abstract

CD56-positive (CD56+) lymphomas, characterized by the expression of the neural cell adhesion molecule on pathological lymphocytes, share a frequent extranodal involvement and a generally aggressive course. Five CD3- CD56+ lymphoma patients presenting with nodular lesions were identified among 180 immunophenotyped cutaneous lymphomas. All the patients were men, with ages ranging from 55 to 78 years. After staging, two patients were diagnosed as having primary cutaneous lymphomas; the remaining three had the secondary cutaneous type. The clinical course was aggressive and four patients died within 8 months from diagnosis. The remaining patient is still alive after a 17-month follow-up. The histological diagnosis was immunoblastic lymphoma in two patients, and medium and large cell pleomorphic lymphoma in three. The angiocentric infiltrate was located mainly in the dermis; azurophilic granules were present in three of the five patients. Immunogenotypic analyses suggested the natural killer cell origin of these neoplasias: all cases exhibited a CD56+ CD3- CD5- T-cell receptor (TCR) silent phenotype, and Southern blot analysis showed a germline configuration of the TCR β-chain gene.

Original languageEnglish
Pages (from-to)966-971
Number of pages6
JournalBritish Journal of Dermatology
Volume137
Issue number6
DOIs
Publication statusPublished - 1997
Externally publishedYes

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