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土耳其儿童伯基特淋巴瘤:临床、病毒学(EBV)及分子研究

Burkitt's lymphoma in Turkish children: clinical, viral [EBV] and molecular studies.

作者信息

Cavdar A O, Yavuz G, Babacan E, Gözdasoglu S, Unal E, Ertem U, Pamir A, Yücesan S, Gökcora H, Uluoglu O

机构信息

Pediatric Hematology and Oncology Research Center, Ankara University, Turkey.

出版信息

Leuk Lymphoma. 1994 Jul;14(3-4):323-30. doi: 10.3109/10428199409049685.

Abstract

Eighty-one Turkish children with Burkitt's lymphoma (BL) were observed during a period of 24 years (1968-1992). The diagnosis was established histologically according to WHO criteria. BL represented 48.5% of NHL in this series. The median age of patients was 5 years with a sex (M/F) ratio of 2.3/1. The most common primary site of tumor involvement at initial presentation was the abdomen (70.4%), which was followed by facial tumors, in particular the jaw and orbit (45.7%). The majority of the patients (84.0%) were in advanced stages (C and D) at initial diagnosis. Facial tumors observed in Turkish children with BL were more similar to African Burkitt's lymphoma than American or European cases. High titers of antibodies against VCA and EA of EBV were also observed in 32 recent cases of BL. Preliminary molecular and immunologic studies revealed EBV-DNA (type I) and T cell deficiency. The clinical presentation, median age, and association with EBV revealed that BL appears to be inbetween African and non-African types in Turkish children. This will be further elucidated in the future by direct examination of tumor cells for EBV and investigation of the molecular characteristics in these cases.

摘要

在24年期间(1968 - 1992年)观察了81例患有伯基特淋巴瘤(BL)的土耳其儿童。根据世界卫生组织标准通过组织学确诊。在该系列中,BL占非霍奇金淋巴瘤的48.5%。患者的中位年龄为5岁,男女比例为2.3/1。初次就诊时肿瘤最常见的原发部位是腹部(70.4%),其次是面部肿瘤,尤其是颌骨和眼眶(45.7%)。大多数患者(84.0%)在初次诊断时处于晚期(C期和D期)。在患有BL的土耳其儿童中观察到的面部肿瘤比美国或欧洲病例更类似于非洲伯基特淋巴瘤。在最近32例BL病例中也观察到针对EBV的VCA和EA的高滴度抗体。初步的分子和免疫学研究显示EBV - DNA(I型)和T细胞缺陷。临床表现、中位年龄以及与EBV的关联表明,在土耳其儿童中BL似乎介于非洲型和非非洲型之间。未来将通过直接检测肿瘤细胞中的EBV以及研究这些病例的分子特征来进一步阐明这一点。

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