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慢性淋巴细胞白血病(CLL)中细胞减少症的发病机制、流行率和预后意义:来自 1518 例 CLL 国家数据库的 213 例患者的回顾性对比研究。

Pathogenesis, prevalence, and prognostic significance of cytopenias in chronic lymphocytic leukemia (CLL): a retrospective comparative study of 213 patients from a national CLL database of 1,518 cases.

机构信息

Kaplan Medical Center, Rehovot, affiliated with Hebrew University Medical School, Jerusalem, Israel.

出版信息

Ann Hematol. 2013 May;92(5):661-7. doi: 10.1007/s00277-012-1663-3. Epub 2012 Dec 29.

DOI:10.1007/s00277-012-1663-3
PMID:23274356
Abstract

Utilizing the database of the Israeli CLL Study Group, we investigated the prevalence and prognostic significance of anemia and thrombocytopenia in patients with chronic lymphocytic leukemia (CLL). Of 1,477 patients, 113 had anemia and thrombocytopenia associated with "infiltrative" marrow failure, median survival of 41 and 86 months, respectively. Autoimmune cytopenias were diagnosed in 100 patients, autoimmune hemolytic anemia (AIHA) in 80, and immune thrombocytopenia (ITP) in 31, while 11 had both co-existent. Median survival of patients with AIHA and ITP, from CLL diagnosis, was 96 and 137 months, respectively, but 29 and 75 months from onset of cytopenia. Patients with AIHA from the time of CLL diagnosis had a significantly shorter survival than those without anemia (p < .0001). Survival was similar for patients with AIHA or anemia due to "infiltrative" bone marrow failure (p = .44). The presence of positive antiglobulin test even without hemolysis was associated with worse outcome. Overall survival of patients with ITP and those without cytopenias (p = 0.94) were similar. In conclusion, laboratory or clinical evidence of AIHA has a significant negative impact on the survival of patients with CLL. Outcome for cases with ITP and patients without cytopenias was similar.

摘要

利用以色列 CLL 研究组的数据库,我们调查了慢性淋巴细胞白血病(CLL)患者贫血和血小板减少症的流行情况及其预后意义。在 1477 名患者中,有 113 名患者伴有“浸润性”骨髓衰竭的贫血和血小板减少症,中位生存时间分别为 41 个月和 86 个月。诊断为自身免疫性血细胞减少症的患者有 100 例,自身免疫性溶血性贫血(AIHA)80 例,免疫性血小板减少症(ITP)31 例,同时存在两种情况的患者有 11 例。从 CLL 诊断开始,AIHA 和 ITP 患者的中位生存时间分别为 96 个月和 137 个月,但从血细胞减少症发病开始分别为 29 个月和 75 个月。从 CLL 诊断开始即患有 AIHA 的患者的生存时间明显短于无贫血的患者(p < .0001)。AIHA 患者与因“浸润性”骨髓衰竭而导致贫血的患者的生存情况相似(p = .44)。即使没有溶血,阳性抗球蛋白试验的存在也与较差的预后相关。有 ITP 和无血细胞减少症患者的总生存率相似(p = 0.94)。总之,AIHA 的实验室或临床证据对 CLL 患者的生存有显著的负面影响。ITP 患者和无血细胞减少症患者的预后相似。

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