Department of Medicine, University of Hong Kong, Hong Kong, Special Administrative Region, People's Republic of China.
Ann Hematol. 2010 Apr;89(4):331-9. doi: 10.1007/s00277-009-0895-3.
To characterize T-cell large granular leukemia in Asia, 22 Chinese patients from a single institute were reported, together with an analysis of 88 Asian and 272 Western patients identified from the literature. In our cohort, anemia due to pure red cell aplasia (PRCA) occurred in 15/22 (68%) of cases, being the most common indication for treatment. Neutropenia was only found in 8/22 (36%) cases, and recurrent infections, the most important clinical problem in Western patients, were not observed. None of our cases presented with rheumatoid arthritis. These clinical features were consistently observed when compared with the 88 other Asian patients. Combined data from our cohort and other Asian cases showed that Asian patients, compared with Western patients, had more frequent anemia (66/110, 60% versus 113/240, 47%; p=0.044), attributable to a much higher incidence of PRCA (52/110, 47% versus 6/143, 4%; p<0.001). However, Western patients presented more frequently than Asian patients with neutropenia (146/235, 62% versus 33/110, 30%; p<0.001) and splenomegaly (99/246, 40% versus 16/110, 15%; p< 0.001). Notably, Western patients were about eight to ten times more likely than Asian patients to have rheumatoid arthritis (73/272, 27% versus 4/106, 4%; p<0.001) and recurrent infections (81/272, 30% versus 3/107, 3%; p<0.001). These clinicopathologic differences have important implications on disease pathogenesis and treatment.
为了描述亚洲的 T 细胞大颗粒淋巴细胞白血病,报道了来自单一机构的 22 例中国患者,并对文献中确定的 88 例亚洲患者和 272 例西方患者进行了分析。在我们的队列中,由于纯红细胞再生障碍性贫血(PRCA)导致的贫血在 22 例中的 15 例(68%)中发生,是治疗的最常见指征。仅在 22 例中的 8 例(36%)中发现中性粒细胞减少症,而在西方患者中最重要的临床问题——反复感染则未观察到。我们的病例均无类风湿关节炎。与其他 88 例亚洲患者相比,这些临床表现一致。来自我们的队列和其他亚洲病例的数据合并表明,与西方患者相比,亚洲患者更常发生贫血(66/110,60%对 113/240,47%;p=0.044),这归因于 PRCA 的发生率高得多(52/110,47%对 6/143,4%;p<0.001)。然而,与亚洲患者相比,西方患者更常出现中性粒细胞减少症(146/235,62%对 33/110,30%;p<0.001)和脾肿大(99/246,40%对 16/110,15%;p<0.001)。值得注意的是,与亚洲患者相比,西方患者患类风湿关节炎(73/272,27%对 4/106,4%;p<0.001)和反复感染(81/272,30%对 3/107,3%;p<0.001)的可能性高 8 至 10 倍。这些临床病理差异对疾病发病机制和治疗具有重要意义。