儿童EBV感染及相关疾病的诊断(讲).ppt

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) 2. High EBV viral load in peripheral blood 3. Clonal expansion of EBV-infected T cells and NK cells 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 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. 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 unknown B cells T/NK cells NK cells T cells 38 27 3 2 12 EBV-infected cells in Japanese patients with CAEBV 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 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.) 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.