Ishihara S, Yabuta R, Tokura Y, Ohshima K, Tagawa S
Section of Pediatrics, Kashiwara Municipal Hospital, Osaka, Japan.
Int J Hematol. 2000 Aug;72(2):223-8.
Recently, we showed that 5 cases of hypersensitivity to mosquito bites (HMB) concealed the clonal lymphoproliferation of Epstein-Barr viral (EBV) DNA-positive natural killer (NK) cells. Although the symptoms of HMB have been supposed to derive from Arthus phenomenon, it has become apparent that this unique disorder has the potential to develop into so-called malignant histiocytosis (MH) or related disorders. Accordingly, the criteria for MH have been changed, and a newer diagnostic name, hemophagocytic syndrome, has been described as being associated with viral infection or leukemia/lymphoma. We previously reported that biopsy specimens taken from skin lesions demonstrated infiltration of lymphocytes bearing the phenotype of NK cells. In this study, we found that skin lesions exhibited infiltration of atypical lymphocytes around the small vessels, resembling angiocentric lymphoma, and that these infiltrating cells were positive for EBER-1 by in situ hybridization. These findings support the concept that HMB is the most important manifestation of a certain type of lymphoproliferative disease that presents with an intense local skin reaction and high fever following mosquito bites, and whose essence is the lymphoproliferation of EBV DNA-positive NK cells.
最近，我们发现5例蚊虫叮咬过敏（HMB）病例隐匿了爱泼斯坦-巴尔病毒（EBV）DNA阳性自然杀伤（NK）细胞的克隆性淋巴细胞增殖。尽管HMB的症状被认为源于阿瑟斯现象，但很明显，这种独特的疾病有可能发展为所谓的恶性组织细胞增多症（MH）或相关疾病。因此，MH的诊断标准已经改变，一种新的诊断名称——噬血细胞综合征，已被描述为与病毒感染或白血病/淋巴瘤相关。我们之前报道过，取自皮肤病变的活检标本显示有具有NK细胞表型的淋巴细胞浸润。在本研究中，我们发现皮肤病变在小血管周围表现出非典型淋巴细胞浸润，类似于血管中心性淋巴瘤，并且通过原位杂交发现这些浸润细胞EBER-1呈阳性。这些发现支持了这样一种概念，即HMB是某种类型的淋巴增殖性疾病的最重要表现，该疾病在蚊虫叮咬后表现为强烈的局部皮肤反应和高热，其实质是EBV DNA阳性NK细胞的淋巴增殖。
