Johnson Steven, Mathews Stephanie, Hudnall S David
Department of Pathology and Laboratory Medicine, The University of North Carolina School of Medicine, Chapel Hill, NC, USA.
Departments of Pathology and Laboratory Medicine, Yale University School of Medicine, New Haven, CT, USA.
Histopathology. 2017 Jun;70(7):1166-1170. doi: 10.1111/his.13157. Epub 2017 Mar 10.
Lymphadenopathy, haematological abnormalities and constitutional symptoms are among the non-specific manifestations seen in drug rash with eosinophilia and systemic symptoms (DRESS), an uncommon but potentially fatal cutaneous adverse drug reaction. The ubiquitous human herpesvirus 6 (HHV-6) plays a unique role in the pathogenesis of DRESS, with emerging data suggesting that reactivation occurs in most cases and contributes to the clinical manifestations, including lymphadenopathy. Further, in the appropriate clinical context, demonstration of HHV-6 reactivation may lend support to a diagnosis of DRESS. The histopathology of DRESS-associated HHV-6 lymphadenitis is reported rarely, with morphologic and immunophenotypic characteristics concerning for T cell lymphoma. The aim is to characterize the histopathology of HHV-6 lymphadenitis in the context of DRESS and to highlight this as an important cause of lymphadenopathy that may be a clinical, morphologic and immunophenotypic mimic of lymphoma.
We describe a case of lymphoma-mimicking lymphadenitis in which the histopathological demonstration of reactivation of HHV-6 infection lent support to the clinical diagnosis of DRESS.
Lymph node biopsies concerning for T cell lymphoma should be evaluated for HHV-6 involvement in a clinical context suggestive of DRESS.
淋巴结病、血液学异常和全身症状是药物性皮疹伴嗜酸性粒细胞增多和全身症状(DRESS)中出现的非特异性表现,DRESS是一种罕见但可能致命的皮肤药物不良反应。普遍存在的人类疱疹病毒6型(HHV-6)在DRESS的发病机制中起独特作用,新出现的数据表明,大多数情况下会发生病毒再激活,并导致包括淋巴结病在内的临床表现。此外,在适当的临床背景下,HHV-6再激活的证明可能支持DRESS的诊断。关于DRESS相关的HHV-6淋巴结炎的组织病理学报道很少,其形态学和免疫表型特征与T细胞淋巴瘤有关。目的是在DRESS背景下描述HHV-6淋巴结炎的组织病理学特征,并强调这是淋巴结病的一个重要原因,可能在临床、形态学和免疫表型上模仿淋巴瘤。
我们描述了一例模仿淋巴瘤的淋巴结炎病例,其中HHV-6感染再激活的组织病理学证明支持了DRESS的临床诊断。
在提示DRESS的临床背景下,对于疑似T细胞淋巴瘤的淋巴结活检应评估是否存在HHV-6感染。