Kwong Y L, Wong K F, Chan L C, Liang R H, Chan J K, Wei D, Chiu E K, Chan C H, Todd D, Chan T K
University Department of Medicine, Queen Mary Hospital, Hong Kong.
Cancer. 1994 Jul 1;74(1):174-81. doi: 10.1002/1097-0142(19940701)74:1<174::aid-cncr2820740128>3.0.co;2-0.
Chronic lymphoproliferative disorders are considered rare in Oriental patients and are thought to constitute only 2% of all leukemias in these patients, compared to 20-30% in Western patients. We conducted a retrospective analysis of Chinese patients with chronic lymphoproliferative disorders to define the frequency and spectrum of these disorders.
A consecutive series of Chinese patients with leukemia and lymphoproliferative disorders seen at two regional hospitals in Hong Kong were analyzed retrospectively. The diagnosis of chronic lymphoproliferative disorders was based on morphologic and immunologic criteria proposed by the French-American-British Cooperative Study Group.
Sixty-four Chinese patients with chronic lymphoproliferative disorders were identified, and these patients constituted 19% of a total of 342 cases of leukemia diagnosed in 3 years. Chronic lymphocytic leukemia was the most common form, occurring at a frequency of 12.5% of all leukemias. The clinicopathologic features of these patients were similar to those of Western patients, except that Chinese patients tended to present with more advanced (Rai's Stages III and IV; Binet's Stage C) and bulky (splenomegaly > 9 cm) disease, and expressed lambda light chain about six times more frequently. Other chronic lymphoproliferative disorders identified in this study included prolymphocytic leukemia, mantle zone lymphoma, hairy cell leukemia, splenic lymphoma with villous lymphocytes, large granular lymphocyte leukemia, and Sezary syndrome. The authors did not identify any case of human T-cell lymphotropic virus-I-related lymphoproliferative disorders within the study period.
In addition to providing the frequencies of various chronic lymphoproliferative disorders in southern Chinese people, this study also showed that these disorders no longer should be considered rare in this population. Inherent biologic differences between lymphoproliferative disorders in Chinese and Western patients also may exist.
慢性淋巴细胞增殖性疾病在东方患者中被认为较为罕见，据认为在这些患者中仅占所有白血病的2%，而在西方患者中这一比例为20% - 30%。我们对中国慢性淋巴细胞增殖性疾病患者进行了回顾性分析，以确定这些疾病的发病率和谱系。
对香港两家地区医院连续收治的中国白血病和淋巴细胞增殖性疾病患者进行回顾性分析。慢性淋巴细胞增殖性疾病的诊断基于法国 - 美国 - 英国合作研究组提出的形态学和免疫学标准。
共确定64例中国慢性淋巴细胞增殖性疾病患者，这些患者占3年内确诊的342例白血病患者总数的19%。慢性淋巴细胞白血病是最常见的类型，占所有白血病的12.5%。这些患者的临床病理特征与西方患者相似，但中国患者往往表现为病情更晚期（Rai分期III和IV期；Binet分期C期）且肿块较大（脾肿大>9 cm），λ轻链表达频率约为西方患者的6倍。本研究中确定的其他慢性淋巴细胞增殖性疾病包括原淋巴细胞白血病、套细胞淋巴瘤、毛细胞白血病、伴绒毛状淋巴细胞的脾淋巴瘤、大颗粒淋巴细胞白血病和Sezary综合征。在研究期间，作者未发现任何与人类T细胞淋巴瘤病毒I相关的淋巴细胞增殖性疾病病例。
除了提供中国南方人群中各种慢性淋巴细胞增殖性疾病的发病率外，本研究还表明，这些疾病在该人群中不再应被视为罕见。中国和西方患者淋巴细胞增殖性疾病之间可能也存在内在生物学差异。
