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非洲伯基特淋巴瘤。历史、生物学、临床特征及治疗

African Burkitt's lymphoma. History, biology, clinical features, and treatment.

作者信息

Magrath I T

机构信息

Pediatric Branch, National Cancer Institute, Bethesda, Maryland 20892.

出版信息

Am J Pediatr Hematol Oncol. 1991 Summer;13(2):222-46.

PMID:2069232
Abstract

Dennis Burkitt's first description of the African tumor that is now known by his name appeared in 1958. In the brief intervening span of 32 years, this lymphoma has provided an extraordinarily valuable paradigm that has afforded insights into topics that encompass the entire discipline of oncology. These include the origins of lymphoid neoplasms at epidemiological, cellular, and molecular levels, and the efficacy of chemotherapy in rapidly progressive, widely disseminated lymphomas. In addition, epidemiological considerations led to the discovery of a new virus, the Epstein-Barr virus, which has proved to be an important human pathogen. This virus probably plays a pathogenetic role in several neoplastic diseases, including the lymphoproliferative syndromes associated with inherited and acquired immunodeficiency. Small, noncleaved cell lymphoma is the latest histological designation of the category of lymphomas that includes Burkitt's lymphoma. This tumor, which is biologically heterogeneous, has become notorious because of its high incidence in individuals infected with the human immunodeficiency virus, which is providing a second, potentially fertile model for the exploration of the pathogenesis of lymphoid neoplasms. Already, enough is known of the pathogenesis of Burkitt's lymphoma to permit the first tentative steps toward the development of novel therapeutic approaches directed toward the molecular genetic abnormalities associated with the neoplasm. In this article, the history, biology, clinical features, and treatment of African Burkitt's lymphoma are reviewed.

摘要

丹尼斯·伯基特(Dennis Burkitt)于1958年首次描述了如今以他的名字命名的非洲肿瘤。在短短32年的时间里,这种淋巴瘤提供了一个极其宝贵的范例,使人们对肿瘤学整个学科所涵盖的主题有了深入了解。这些主题包括淋巴肿瘤在流行病学、细胞和分子水平上的起源,以及化疗在快速进展、广泛播散的淋巴瘤中的疗效。此外,流行病学方面的考虑促使发现了一种新病毒——爱泼斯坦-巴尔病毒(Epstein-Barr virus),事实证明它是一种重要的人类病原体。这种病毒可能在几种肿瘤性疾病中发挥致病作用,包括与遗传性和获得性免疫缺陷相关的淋巴增殖综合征。小无裂细胞淋巴瘤是包括伯基特淋巴瘤在内的淋巴瘤类别的最新组织学名称。这种生物学上具有异质性的肿瘤因其在感染人类免疫缺陷病毒的个体中高发而声名狼藉,这为探索淋巴肿瘤的发病机制提供了第二个潜在的丰富模型。目前,对于伯基特淋巴瘤的发病机制已经有了足够的了解,从而能够朝着开发针对与该肿瘤相关的分子遗传异常的新型治疗方法迈出初步尝试性的步伐。本文将对非洲伯基特淋巴瘤的历史、生物学、临床特征及治疗进行综述。

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