Stetsenko Galina Y, McFarlane Rob, Kalus Andrea, Olerud John, Cherian Sindhu, Fromm Jonathan, George Evan, Argenyi Zsolt
Department of Pathology, University of Washington, Seattle, WA 98195-6100, USA.
J Cutan Pathol. 2008 Jun;35(6):579-84. doi: 10.1111/j.1600-0560.2007.00845.x. Epub 2007 Nov 12.
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.
CD4+/CD56+血液皮肤肿瘤（HN），以前称为母细胞性自然杀伤（NK）细胞淋巴瘤，是一种罕见的皮肤树突状细胞肿瘤亚型，以高度侵袭性行为为特征。其特征包括：T辅助/诱导细胞标志物CD4和NK细胞标志物CD56表达，而无其他T细胞或NK细胞特异性标志物。特别是，不表达CD3（表面或细胞质）和CD2。虽然一般报道T细胞受体（TCR）基因呈种系构型，但我们报告了一例CD4+/CD56+ HN的不寻常变异型，其TCR基因发生了克隆重排。CD4+/CD56+ HN的这一特征仅有极少报道。认识到一小部分CD4+/CD56+ HN中存在克隆性TCR基因重排对于避免将该实体误诊为皮肤T细胞淋巴瘤的不寻常变异型很重要。
