肯尼亚三个地区伯基特淋巴瘤的临床特征

Clinical characteristics of Burkitt's lymphoma from three regions in Kenya.

作者信息

Mwanda W O, Orem J, Remick S C, Rochford R, Whalen C, Wilson M L

机构信息

Department of Haematology and Blood Transfusion, Kenyatta National Hospital and the University of Nairobi College of Health Sciences, Kenya.

出版信息

East Afr Med J. 2005 Sep;82(9 Suppl):S135-43. doi: 10.4314/eamj.v82i9.9385.

OBJECTIVES

To describe the clinical characteristics of Burkitt's lymphoma (BL) from three regions in Kenya at different altitudes with a view towards understanding the contribution of local environmental factors.

DESIGN

Prospective cross-sectional study.

SETTING

Kenyatta National Hospital and seven provincial hospitals in Kenya.

METHOD

Histologically proven cases of Burkitt's lymphoma in patients less than 16 years of age were clinically examined and investigated.

MAIN OUTCOME MEASURES

For every case the following parameters were documented: chief complaint(s); physical examination, specifically pallor, jaundice, oedema, lymphadenopathy, presence of masses, splenomegaly and hepatomegaly. Reports of evaluation of chest radiograph, abdominal ultrasound/scan, bone marrow aspiration, cerebral spinal fluid cytology, liver and kidney function tests, urinalysis, stool occult blood and full blood count results. Stage of disease was assigned A, B, C or D. Cases of BL from three provinces of Kenya with diverse geographical features were analysed: Central, Coast, and Western.

RESULTS

This study documented 471 BL cases distributed as follows: Central 61 (males 39 and 22 females), M:F ratio 1.8:1; Coast 169 (111 males and 58 females), M:F ratio 1.9:1; and Western 241 (140 males and 101 females), M:F ratio 1.4:1. The major presenting complaints were: abdominal swelling--Central 36%, Coast 4% and Western 26%; swelling on the face--Central 31%, Coast 81% and Western 64%; and proptosis--Central 3%, Coast 1% and Western 9%. The mean duration of these complaints in weeks were Central 6.9, Coast 6.08, and Western 5.05. The initial physical finding was a tumour mass in 39%, 72% and 54% of cases for Central, Coast and Western respectively. Tumour stage at diagnosis was: stage A--Central 21%, Coast 43% and Western 34%; stage B--Central 10%, Coast 5% and Western 10%; stage C--Central 41%, Coast 34% and Western 30%; and stage D--Central 28%, Coast 17% and Western 26%. For the age and sex matched cases the results show that commonly involved sites were: abdomen--Central 35%, Coast 9% and Western 14%; jaw (mandible)--Central 24%, Coast 22% and Western 31%; maxilla--Central 6%, Coast 24% and Western 11%; and lymph nodes--Central 10%, Coast 4% and Western 8%. The disease stage was A--Central 33%, Coast 44% and Western 36%; stage B--Central 11%, Coast 10% and Western 27%; stage C--Central 39%, Coast 34% and Western 27%; and stage D--Central 21%, Coast 13% and Western 37%.

CONCLUSION

This study shows that clinical features of childhood BL vary with geographical region. The variations are documented in proportion of jaw, maxilla, abdominal and lymph nodal sites involvement. The differences observed are potentially due to the local environmental factors within these provinces. BL cases from Western province had features, intermediate between endemic and sporadic. Coastal province BL cases were similar to endemic BL, while BL cases from Central province resembled more or less sporadic BL subtypes. Strategies to explain and investigate the local environmental factors associated with the observed differences may certainly contribute towards improved understanding and clinical management of BL.

目的

描述肯尼亚不同海拔三个地区伯基特淋巴瘤（BL）的临床特征，以了解当地环境因素的影响。

设计

前瞻性横断面研究。

地点

肯尼亚肯雅塔国家医院和七家省级医院。

方法

对16岁以下经组织学确诊的伯基特淋巴瘤患者进行临床检查和调查。

主要观察指标

记录每例患者的以下参数：主要症状；体格检查，特别是面色苍白、黄疸、水肿、淋巴结病、肿块、脾肿大和肝肿大情况。胸部X光、腹部超声/扫描、骨髓穿刺、脑脊液细胞学检查、肝肾功能检查、尿液分析、粪便潜血检查和全血细胞计数结果的报告。疾病分期分为A、B、C或D期。分析了肯尼亚三个地理特征不同省份的BL病例：中部、沿海和西部。

结果

本研究记录了471例BL病例，分布如下：中部61例（男性39例，女性22例），男女性别比为1.8:1；沿海169例（男性111例，女性58例），男女性别比为1.9:1；西部241例（男性140例，女性101例），男女性别比为1.4:1。主要症状如下：腹部肿胀——中部36%，沿海4%，西部26%；面部肿胀——中部31%，沿海81%，西部64%；眼球突出——中部3%，沿海1%，西部9%。这些症状的平均持续时间（以周计）分别为：中部6.9周，沿海6.08周，西部5.05周。最初的体格检查发现肿瘤肿块的比例分别为：中部39%，沿海72%，西部54%。诊断时的肿瘤分期为：A期——中部21%，沿海43%，西部34%；B期——中部10%，沿海5%，西部10%；C期——中部41%，沿海34%，西部30%；D期——中部28%，沿海17%，西部26%。对于年龄和性别匹配的病例，结果显示常见受累部位为：腹部——中部35%，沿海9%，西部14%；颌骨（下颌骨）——中部24%，沿海22%，西部31%；上颌骨——中部6%，沿海24%，西部11%；淋巴结——中部10%，沿海4%，西部8%。疾病分期为：A期——中部33%，沿海44%，西部36%；B期——中部11%，沿海10%，西部27%；C期——中部39%，沿海34%，西部27%；D期——中部21%，沿海13%，西部37%。

结论

本研究表明儿童BL的临床特征因地理区域而异。这种差异记录在颌骨、上颌骨、腹部和淋巴结受累部位的比例上。观察到的差异可能是由于这些省份的当地环境因素。西部省份的BL病例具有介于地方性和散发性之间的特征。沿海省份的BL病例与地方性BL相似，而中部省份的BL病例或多或少类似于散发性BL亚型。解释和研究与观察到的差异相关的当地环境因素的策略肯定有助于更好地理解和临床管理BL。