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土耳其儿童中非洲型和美国型伯基特淋巴瘤的临床、病毒学(EB病毒)及分子研究

Burkitt's lymphoma between African and American types in Turkish children: clinical, viral (EBV), and molecular studies.

作者信息

Cavdar A O, Gözdaşoğlu S, Yavuz G, Babacan E, Unal E, Uluoğlu O, Yücesan S, Magrath I T, Akar N

机构信息

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

出版信息

Med Pediatr Oncol. 1993;21(1):36-42. doi: 10.1002/mpo.2950210108.

Abstract

Seventy-two Turkish children with Burkitt's lymphoma (BL) observed during a period of 22 years (1968-1990) have been analysed retrospectively. The diagnosis was established histologically according to WHO criteria. BL represented 50% of NHL in this series. The patients were staged according to Ziegler's system. The median age of patients was 5.5 years with a sex (M/F) ratio of 2.1/1. The most common primary site of tumor involvement was the abdomen (69.4%), which was followed by facial tumors, in particular the jaw and orbit (49.9%). There were 21 cases with jaw (29.1%) and 15 cases with orbital involvement (20.8%) at initial presentation. The majority of the patients (84.4%) 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 seen in our recent cases of BL. Two main treatment regimens, namely, single agent chemotherapy with cyclophosphamide (CYX) (1968-1974) and three drug (COM) combination chemotherapy, were used consecutively (1974-1988). COM has been shown to produce better results than single agent therapy. The clinical presentation, mean age, and high antibodies (IgG) to EBV and preliminary molecular studies revealed that BL appears to be in between African and non-African types in Turkish children. This will be further elucidated by direct examination of tumor cells for EBV and investigation of the molecular characteristics of Turkish tumors. Such studies are presently under way.

摘要

对1968年至1990年这22年间观察到的72例土耳其伯基特淋巴瘤(BL)患儿进行了回顾性分析。根据世界卫生组织标准通过组织学确诊。在本系列中,BL占非霍奇金淋巴瘤(NHL)的50%。根据齐格勒系统对患者进行分期。患者的中位年龄为5.5岁,男女比例为2.1/1。肿瘤累及的最常见原发部位是腹部(69.4%),其次是面部肿瘤,尤其是颌骨和眼眶(49.9%)。初诊时,有21例颌骨受累(29.1%),15例眼眶受累(20.8%)。大多数患者(84.4%)在初诊时处于晚期(C期和D期)。与美国或欧洲病例相比,土耳其BL患儿中观察到的面部肿瘤与非洲伯基特淋巴瘤更为相似。在我们最近的BL病例中也发现了针对EBV的VCA和EA的高滴度抗体。先后使用了两种主要治疗方案,即环磷酰胺(CYX)单药化疗(1968 - 1974年)和三药联合化疗(COM)(1974 - 1988年)。已证明COM比单药治疗效果更好。临床表现、平均年龄、对EBV的高抗体(IgG)以及初步分子研究表明,土耳其儿童的BL似乎介于非洲型和非非洲型之间。通过直接检查肿瘤细胞中的EBV以及研究土耳其肿瘤的分子特征将进一步阐明这一点。此类研究目前正在进行中。

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