Merino F, Henle W, Ramírez-Duque P
J Clin Immunol. 1986 Jul;6(4):299-305. doi: 10.1007/BF00917330.
The results of clinical and Epstein-Barr virus (EBV) serological studies on nine Chediak-Higashi syndrome (CHS) patients are reported. Persistently elevated antibodies to the viral capsid antigen (VCA) and the restricted component of the early antigen complex (EA-R) developed in six patients who experienced primary EBV infection which either remained silent or were accompanied by clinical signs of infectious mononucleosis (IM). Hepatosplenomegaly and moderate lymphadenopathy, both clinical signs of the accelerated phase, remained detectable in the six patients for a long period of time after seroconversion. The clinical, serological, and histopathological observations are suggestive of a nonmalignant lymphoproliferative disease and consistent with an immunodeficiency to EBV. The abnormal serological responses to EBV in CHS are therefore considered manifestations of a chronic active EBV infection which may result in lethal lymphoproliferation. The three as yet seronegative CHS patients revealed no signs of the accelerated lymphoproliferative phase of the syndrome.
本文报告了对9例Chediak-Higashi综合征（CHS）患者进行临床及爱泼斯坦-巴尔病毒（EBV）血清学研究的结果。6例经历原发性EBV感染的患者，其病毒衣壳抗原（VCA）抗体和早期抗原复合物（EA-R）的受限成分持续升高，这些感染要么处于潜伏状态，要么伴有传染性单核细胞增多症（IM）的临床症状。肝脾肿大和中度淋巴结病这两个加速期的临床症状，在血清转换后的很长一段时间内，在这6例患者中仍可检测到。临床、血清学和组织病理学观察结果提示为一种非恶性淋巴增殖性疾病，且与对EBV的免疫缺陷一致。因此，CHS患者对EBV的异常血清学反应被认为是慢性活动性EBV感染的表现，这可能导致致命的淋巴增殖。3例血清学仍为阴性的CHS患者未出现该综合征加速性淋巴增殖期的迹象。
