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Indolent CD8+ lymphoid proliferation of the ear: A phenotypic variant of the small‐medium pleomorphic cutaneous T‐cell lymphoma?
Author(s) -
Beltraminelli Helmut,
Müllegger Robert,
Cerroni Lorenzo
Publication year - 2010
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.2009.01278.x
Subject(s) - lymphoma , pathology , cd8 , cytotoxic t cell , phenotype , gene rearrangement , immunophenotyping , medicine , cutaneous t cell lymphoma , dermis , peripheral t cell lymphoma , immunohistochemistry , biology , t cell , mycosis fungoides , immunology , antigen , gene , immune system , in vitro , biochemistry
Background: Recently, Petrella et al. described four patients with an unusual CD8+ lymphoid proliferation arising on the ear. These cases do not correspond clearly to any recognized category of cutaneous T‐cell lymphoma (CTCL) described in the World Health Organization (WHO)/European Organization for Research and Treatment of Cancer (EORTC) 2005 classification. Methods and Results: Three patients (all men; median age 64; range: 61‐69) presented with plaques or small tumors localized on the ears. All lesions showed histopathologically a dense, diffuse infiltration of lymphocytes within the entire dermis without epidermotropism. Cytomorphology revealed predominance of medium‐sized pleomorphic lymphocytes. Immunohistochemistry showed a cytotoxic phenotype (CD3 + /CD4 −/CD8 +). Polymerase chain reaction (PCR) analysis of the T‐cell receptor (TCR)‐gamma gene revealed a monoclonal rearrangement in two of three patients. Follow‐up data of two patients were available; one is alive without skin or systemic manifestations of the disease after 28 months, whereas the other is alive with persistent skin disease after 7 months. Conclusions: Our observation confirms that some patients present with a peculiar lymphoid proliferation of small‐medium pleomorphic cytotoxic lymphocytes located on the ear, probably representing a phenotypic variant of the cutaneous small/medium pleomorphic T‐cell lymphoma (CSMPTCL). These cases should not be misinterpreted as a high‐grade cytotoxic lymphoma.