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阿尔及利亚的腹部伯基特型淋巴瘤。

Abdominal Burkitt-type lymphomas in Algeria.

作者信息

Ladjadj Y, Philip T, Lenoir G M, Tazerout F Z, Bendisari K, Boukheloua R, Biron P, Brunat-Mentigny M, Aboulola M

出版信息

Br J Cancer. 1984 Apr;49(4):503-12. doi: 10.1038/bjc.1984.78.

Abstract

In a previous retrospective analysis from the principal paediatric centres of Algeria, Burkitt-type lymphomas (BL) were shown to account for around 46.5% of the total childhood non-Hodgkin's malignant lymphomas in that country. In the present study, a series of 49 abdominal BL from the Paediatric Clinic of Surgery, Mustapha Hospital, Algiers, has been studied. The age distribution shows a peak between 4 and 5 years of age, and the sex ratio is (M:F) 2.26:1. The disease is characterized by a rapid evolution in the absence of therapy. The major problem is an explosive form of the disease, which at present seems difficult to control in this country. Fifteen of the 49 patients (30.6%) died before completion of the first course of chemotherapy; however, complete remission (CR) was obtained for 30 patients (61%). Overall survival was 42.85% (21/49), whereas survival of patients who reached CR is 70% (21/30). When CR was obtained, deaths were related to cerebrospinal fluid involvement, local recurrence, secondary bone marrow involvement or therapeutic accidents. All patients alive with no evidence of disease (NED) 8-months after CR can be considered definitively cured. Epstein-Barr virus (EBV) serology performed on 31 BL patients and on a control group of 25 children with other malignant tumours showed that most Algerian BL have elevated EBV titres. A search for viral markers within malignant cells in 17 patients indicated that 88% (15/17) of the BL cases were EBV-associated. Analysis of the immunological and cytogenetic data showed that, as in the rest of the world, these BL cases involve proliferation of B-cell-type lymphocytes, with characteristic cytogenetic translocations involving chromosome 8. This report represents the most detailed description so far of BL from an area in non-equatorial Africa and the first report of a large series from North Africa.

摘要

在先前一项来自阿尔及利亚主要儿科中心的回顾性分析中,伯基特型淋巴瘤(BL)占该国儿童非霍奇金恶性淋巴瘤总数的约46.5%。在本研究中,对阿尔及尔穆斯塔法医院小儿外科诊所的49例腹部BL病例进行了研究。年龄分布显示在4至5岁之间出现峰值,性别比为(男:女)2.26:1。该疾病的特点是在未经治疗的情况下迅速发展。主要问题是疾病的爆发形式,目前在该国似乎难以控制。49例患者中有15例(30.6%)在完成第一个化疗疗程前死亡;然而,30例患者(61%)获得了完全缓解(CR)。总生存率为42.85%(21/49),而达到CR的患者生存率为70%(21/30)。当获得CR时,死亡与脑脊液受累、局部复发、继发性骨髓受累或治疗意外有关。所有在CR后8个月无疾病证据(NED)存活的患者可被视为已彻底治愈。对31例BL患者和25例患有其他恶性肿瘤的儿童对照组进行的爱泼斯坦-巴尔病毒(EBV)血清学检测表明,大多数阿尔及利亚BL患者的EBV滴度升高。对17例患者恶性细胞内病毒标志物的检测表明,88%(15/17)的BL病例与EBV相关。免疫和细胞遗传学数据分析表明,与世界其他地区一样,这些BL病例涉及B细胞型淋巴细胞增殖,伴有涉及8号染色体的特征性细胞遗传学易位。本报告是迄今为止对来自非洲非赤道地区的BL最详细的描述,也是来自北非的大量病例的首次报告。

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