Ogata Masao
Department of Hematology, Oita University Faculty of Medicine, Yufu-city, Oita, Japan.
J Clin Exp Hematop. 2009 Nov;49(2):57-67. doi: 10.3960/jslrt.49.57.
Pathogenetic roles of human herpesvirus (HHV)-6 in lymphoproliferative diseases have been of continued interest. Many molecular studies have tried to establish a pathogenic role for HHV-6 in lymphoid malignancies. However, whether HHV-6 plays a role in these pathologies remains unclear, as positive polymerase chain reaction results for HHV-6 in those studies may reflect latent infection or reactivation rather than presence of HHV-6 in neoplastic cells. A small number of studies have investigated HHV-6 antigen expression in pathologic specimens. As a result, the lack of HHV-6 antigen expression on neoplastic cells argues against any major pathogenic role of HHV-6. The role of HHV-6 in childhood acute lymphoblastic leukemia (ALL) has also been of interest but remains controversial, with 2 studies documenting higher levels of HHV-6 antibody in ALL patients, and another 2 large-scale studies finding no significant differences in HHV-6 seroprevalences between ALL patients and controls. Alternatively, HHV-6 is increasingly recognized as an important opportunistic pathogen. HHV-6 reactivation is common among recipients of allogeneic stem cell transplantation (SCT), and is linked to various clinical manifestations. In particular, HHV-6 encephalitis appears to be significant, life-threatening complication. Most HHV-6 encephalitis develops in patients receiving transplant from an unrelated donor, particularly cord blood, typically around the time of engraftment. Symptoms are characterized by short-term memory loss and seizures. Magnetic resonance imaging typically shows limbic encephalitis. Prognosis for HHV-6 encephalitis is poor, but appropriate prophylactic measures have not been established. Establishment of preventive strategies against HHV-6 encephalitis represents an important challenge for physicians involved with SCT.
人类疱疹病毒6型(HHV-6)在淋巴增殖性疾病中的致病作用一直备受关注。许多分子研究试图确立HHV-6在淋巴系统恶性肿瘤中的致病作用。然而,HHV-6是否在这些病变中发挥作用仍不清楚,因为那些研究中HHV-6聚合酶链反应阳性结果可能反映的是潜伏感染或病毒再激活,而非肿瘤细胞中存在HHV-6。少数研究调查了病理标本中HHV-6抗原的表达情况。结果显示,肿瘤细胞上缺乏HHV-6抗原表达,这与HHV-6的任何主要致病作用相悖。HHV-6在儿童急性淋巴细胞白血病(ALL)中的作用也备受关注,但仍存在争议,有两项研究记录了ALL患者中HHV-6抗体水平较高,而另外两项大规模研究发现ALL患者与对照组之间HHV-6血清阳性率无显著差异。另外,HHV-6越来越被认为是一种重要的机会性病原体。HHV-6再激活在异基因干细胞移植(SCT)受者中很常见,并与各种临床表现相关。特别是,HHV-6脑炎似乎是一种严重的、危及生命的并发症。大多数HHV-6脑炎发生在接受无关供者移植的患者中,尤其是脐血移植患者,通常在植入前后。症状表现为短期记忆丧失和癫痫发作。磁共振成像通常显示为边缘叶脑炎。HHV-6脑炎的预后很差,但尚未确立适当的预防措施。制定针对HHV-6脑炎的预防策略对从事SCT的医生来说是一项重要挑战。