CD4+/CD56+ hematodermic neoplasm: report of a rare variant with a T-cell receptor gene rearrangement.
J Cutan Pathol
; 35(6): 579-84, 2008 Jun.
Article
in En
| MEDLINE
| ID: mdl-18005171
ABSTRACT
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.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Skin Neoplasms
/
CD4 Antigens
/
Lymphoma, T-Cell, Cutaneous
/
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
/
CD56 Antigen
Limits:
Humans
/
Male
/
Middle aged
Language:
En
Journal:
J Cutan Pathol
Year:
2008
Type:
Article
Affiliation country:
United States