Shvidel L, Shtarlid M, Klepfish A, Sigler E, Berrebi A
Hematology Institute, Kaplan Medical Center, Hebrew University, Faculty of Medicine, Rehovot, Israel.
Leukemia. 1998 Oct;12(10):1612-7. doi: 10.1038/sj.leu.2401140.
Chronic lymphocytic leukemia (CLL) represents 30% of all leukemias in Caucasians. In East Europe and USA the disease incidence is high while in Asia and Africa CLL is rare. The present study deals with 302 cases of B cell CLL and related disorders; 207 patients originating from Europe and America (Ashkenazi Jews) and 95 descendants from Asia, The Mediterranean or Africa (Sephardic Jews). The patients were recruited during 1975-1996 in a single center covering the Hashfela region -- a Southern area of Israel with a current population of 430000 inhabitants. Incidence of the disease, clinical pattern, biological parameters, prognosis and outcome were investigated and compared in both ethnic groups. The results of this study show a high incidence of CLL in Israel. The mean annual age-adjusted incidence 4.3 per 100000 person-year is among the highest reported values. Our study confirms previous data on the prevalence of CLL in Ashkenazi compared to Sephardic Jews. The rise in CLL rate in the reviewed period occurred in both populations, mainly in the Sephardic group. The relative risk for Ashkenazies compared to Sephardics decreased from 6.0 in the 1975-1979 period to 2.4 in 1990-1996. A high rate of CLL was found in new immigrants from the former USSR with 26 cases de novo diagnosed and 11 prevalent cases not included in this series among approximately 60000 new immigrants in the ara over the last 8 years. No differences were found in clinical, laboratory and immunological parameters at the time of diagnosis in the two ethnic groups. The follow-up showed a similar pattern in the disease evolution. A preliminary study of immunoglobulin heavy chain rearrangement performed in 14 patients showed no significant differences in JH hybridization in the early stages of the disease, but more aberrations in advanced CLL in the Ashkenazi group. Our findings suggest that ethnic origin of the patients itself does not affect the biological and clinical behavior of this disease.
慢性淋巴细胞白血病(CLL)占白种人所有白血病病例的30%。在东欧和美国,该病发病率较高,而在亚洲和非洲,CLL较为罕见。本研究涉及302例B细胞CLL及相关疾病;207例患者来自欧美(阿什肯纳兹犹太人),95例为亚洲、地中海地区或非洲后裔(塞法迪犹太人)。这些患者于1975年至1996年期间在一个覆盖哈什费拉地区的单一中心招募,该地区是以色列南部地区,目前有43万居民。对两个种族群体的疾病发病率、临床模式、生物学参数、预后和结局进行了调查和比较。本研究结果显示以色列CLL发病率较高。年龄调整后的年平均发病率为每10万人年4.3例,是报告的最高值之一。我们的研究证实了先前关于阿什肯纳兹犹太人相较于塞法迪犹太人中CLL患病率的数据。在回顾期内,两个群体的CLL发病率均有所上升,主要是塞法迪群体。与塞法迪人相比,阿什肯纳兹人的相对风险从1975 - 1979年期间的6.0降至1990 - 1996年的2.4。在过去8年中,在约60000名来自前苏联的新移民中,发现了较高的CLL发病率,有26例新发诊断病例,本系列未纳入11例现患病例。在诊断时,两个种族群体的临床、实验室和免疫学参数未发现差异。随访显示疾病演变模式相似。对14例患者进行的免疫球蛋白重链重排初步研究表明,在疾病早期,JH杂交在两个群体中无显著差异,但在阿什肯纳兹群体的晚期CLL中存在更多异常。我们的研究结果表明,患者的种族本身并不影响该疾病的生物学和临床行为。