Ohshima K, Shimazaki K, Sugihara M, Haraoka S, Suzumiya J, Kanda M, Kawasaki C, Kikuchi M
Department of Pathology, School of Medicine, Fukuoka University, Nanakuma, Japan.
Pathol Int. 1999 Jun;49(6):533-40. doi: 10.1046/j.1440-1827.1999.00921.x.
Non-neoplastic hemophagocytic syndrome (HPS), also called virus-associated hemophagocytic syndrome (VAHS), has been thought to be a distinct clinical entity. A spontaneous recovery is common, but the prognosis of Epstein-Barr virus (EBV)-associated VAHS is poor. However, the role of EBV has yet to be clearly elucidated. A retrospective study of the bone marrow of 30 cases, in which the diagnosis of non-neoplastic VAHS was clinicopathologically confirmed, was performed. We were unable to histologically confirm the presence of neoplastic lesions, especially lymphoma cell infiltration. Ten of the patients were children (aged less than 15 years) and young adults (aged under 20 years; median age, 10 years). Twenty patients were adults (aged over 21 years; median age, 48 years). Twelve of these patients died, while 18 showed a spontaneous recovery. We performed immunological staining and in situ hybridization (ISH) for EBV. To clarify the presence of apoptosis, an in situ apoptosis detection (tunnel) method was used. In situ hydridization showed an EBV-presence in 16 of the 30 patients. In addition, the EBV-presence was confined in the lymphocytes, especially T lymphocytes in double stainings. The number of EBV-infected cells varied; however, the EBV presence was associated with ages. Nine of the 10 children and young adults showed an EBV-presence, while EBV was detected in seven of the 20 adults. Especially in 10 patients aged over 49 years, no EBV was detected. According to the in situ apoptosis detection, apoptotic cells were increased in number and considered to be lymphoid cells, but not myeloid or histiocytic cells. Some apoptotic cells were phagocyted with histiocytes. Histologically, apoptosis may be one of the factors that induced phagocytosis.
非肿瘤性噬血细胞综合征（HPS），也称为病毒相关性噬血细胞综合征（VAHS），一直被认为是一种独特的临床实体。自发缓解很常见，但与 Epstein-Barr 病毒（EBV）相关的 VAHS 预后较差。然而，EBV 的作用尚未明确阐明。我们对 30 例经临床病理确诊为非肿瘤性 VAHS 的骨髓进行了回顾性研究。我们无法在组织学上证实存在肿瘤性病变，尤其是淋巴瘤细胞浸润。其中 10 例患者为儿童（年龄小于 15 岁）和青年（年龄小于 20 岁；中位年龄为 10 岁）。20 例患者为成年人（年龄超过 21 岁；中位年龄为 48 岁）。这些患者中有 12 例死亡，18 例自发缓解。我们对 EBV 进行了免疫染色和原位杂交（ISH）。为了阐明凋亡的存在，使用了原位凋亡检测（隧道）方法。原位杂交显示 30 例患者中有 16 例存在 EBV。此外，在双重染色中，EBV 的存在局限于淋巴细胞，尤其是 T 淋巴细胞。EBV 感染细胞的数量各不相同；然而，EBV 的存在与年龄有关。10 例儿童和青年中有 9 例存在 EBV，而 20 例成年人中有 7 例检测到 EBV。特别是在 10 例年龄超过 49 岁的患者中，未检测到 EBV。根据原位凋亡检测，凋亡细胞数量增加，且被认为是淋巴细胞，而非髓细胞或组织细胞。一些凋亡细胞被组织细胞吞噬。在组织学上，凋亡可能是诱导吞噬作用的因素之一。
