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CD4+/CD56+ hematodermic neoplasm: report of a rare variant with a T‐cell receptor gene rearrangement
Author(s) -
Stetsenko Galina Y,
McFarlane Rob,
Kalus Andrea,
Olerud John,
Cherian Sindhu,
Fromm Jonathan,
George Evan,
Argenyi Zsolt
Publication year - 2008
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.1600-0560.2007.00845.x
Subject(s) - t cell receptor , gene rearrangement , cd3 , biology , t cell lymphoma , lymphoma , t cell , neoplasm , gene , cd8 , antigen , immunology , genetics , immune system
CD4+/CD56+ hematodermic neoplasm (HN), formerly known as a blastic natural killer (NK) cell lymphoma, is a rare subtype of a cutaneous dendritic cell neoplasm notable for highly aggressive behavior. The characteristic features are: expression of the T‐helper/inducer cell marker CD4 and the NK‐cell marker CD56 in the absence of other T cell or NK‐cell specific markers. In particular, CD3 (surface or cytoplasmic) and CD2 are not expressed. Although T‐cell receptor (TCR) genes are generally reported to be in a germline configuration, we present an unusual variant of a CD4+/CD56+ HN with a clonal rearrangement of TCR genes. This feature of a CD4+/CD56+ HN has been only rarely reported. Recognition of the presence of clonal TCR gene rearrangements in a small subset of CD4+/CD56+ HN is important to avoid misdiagnosis of this entity as an unusual variant of a cutaneous T‐cell lymphoma.