Buchwald D, Freedman A S, Ablashi D V, Sullivan J L, Caligiuri M, Weinberg D S, Hall C G, Ashley R L, Saxinger C, Balachandran N
University of Washington School of Medicine, Seattle 98121.
J Clin Immunol. 1990 Nov;10(6):335-44. doi: 10.1007/BF00917479.
A 17-year-old, previously healthy woman developed an acute "mononucleosis-like" illness with an associated "atypical" pneumonitis, followed by years of debilitating chronic fatigue, fevers, a 10-kg weight loss, night sweats, and neurocognitive symptoms. Thereafter, her sister developed a similar but less severe illness. The patient developed marked, chronic lymphadenopathy and splenomegaly, with associated persistent relative lymphocytosis and atypical lymphocytosis and with thrombocytopenia. After 3 years of illness, a splenectomy was performed, which resulted in some symptomatic improvement, prompt weight gain, and resolution of all hematologic abnormalities. Serial immunologic studies revealed a strikingly elevated number of activated B lymphocytes and a T lymphopenia, which improved but did not return to normal postsplenectomy. No causal association was found with any of several infectious agents that could produce such a lymphoproliferative illness. However, both the patient and her sister had evidence of active infection with the recently discovered human herpesvirus-6. Seven years after the onset of the illness, the patient and her sister remain chronically ill.
一名17岁、此前身体健康的女性患上了一种急性“传染性单核细胞增多症样”疾病,并伴有“非典型”肺炎,随后多年一直饱受慢性疲劳、发热、体重减轻10公斤、盗汗及神经认知症状的折磨。此后,她的妹妹也患上了类似但症状较轻的疾病。该患者出现了明显的慢性淋巴结病和脾肿大,伴有持续性相对淋巴细胞增多和非典型淋巴细胞增多以及血小板减少。患病3年后,患者接受了脾切除术,术后症状有所改善,体重迅速增加,所有血液学异常均得到缓解。系列免疫学研究显示,活化B淋巴细胞数量显著升高,T淋巴细胞减少,脾切除术后有所改善但未恢复正常。未发现与任何可导致此类淋巴细胞增生性疾病的感染因子存在因果关联。然而,患者及其妹妹均有新近发现的人类疱疹病毒6型的活动性感染证据。患病7年后,患者及其妹妹仍患有慢性病。