儿童感染及相关疾病的诊断讲谢正.pptVIP

慢性活动性EBV感染 第三十页，共58页 CAEBV is characterized by severe, chronic or recurrent infectious mononucleosis-like symptoms after a primary EBV infection, and has a high morbidity and mortality from hepatic failure, lymphoma, sepsis, or hemophagocytic syndrome. 1. Unusual pattern of anti-EBV antibodies (high levels of IgG anti-VCA and EA, absence of anti-EBNA) High EBV viral load in peripheral blood Clonal expansion of EBV-infected T cells and NK cells 第三十一页，共58页 Historical milestones of CAEBV 1948, Issacs: prolonged fever, malaise, lymphadenopathy, hepatosplenomegaly 1975, Horwitz et al:such clinical manifestations with mildly or moderately high or positive IgG against VCA and EA 1982, Tobi et al: similar atypical illness associated with serological evidence of persistent EBV infection 第三十二页，共58页 Historical milestones of CAEBV 1984, Dubois et al: criteria for such cases termed chronic mononucleosis syndrome:(1) disabling fatigue and malaise;(2) low-grade afternoon fever;(3) variable other nonspecific symptoms: myalgias, sore throat, depression,lasting 6 months or longer, with EBV serologies of (1)VCA-IgG?160, (2)EA-IgG ?5, (3) postive anti-EBNA, (4)absent VCA-IgM, (5) absent Paul-Bunnell heterophil antibody. 第三十三页，共58页 发病机制 EBV感染的T细胞或NK细胞克隆性增殖 存在的问题： 1.如何感染T细胞或NK细胞 2.如何引起临床症状 第三十四页，共58页 EBV-infected cells in Japanese patients with CAEBV 第三十五页，共58页 临床表现 发热：间断性发热 淋巴结肿大 肝脾肿大 间质性肺炎 贫血 肝炎 眼葡萄膜炎 第三十六页，共58页 第三十七页，共58页 第三十八页，共58页 Diagnostic criteria of CAEBV I. Severe illness of greater than 6 months’ duration that: 1. Began as primary EBV infection OR 2. Is associated with grossly abnormal EBV antibody titers (IgG to VCA≥1:5,120; antibody to EA≥1:640; or antibody to EBNA＜1:2), AND (Straus S.E.) 第三十九页，共58页 II. Histological evidence of major organ involvement, such as: 1. Interstitial pneumonia 2. Hypoplasia of some bone marrow elements 3. Uveitis 4. Lymphadenitis 5. Persistent hepatitis 6. Splenomegaly, AND III. Detection of increased quantities of EBV in affecte