Autoimmune cytopenia does not predict poor prognosis in chronic lymphocytic leukemia/small lymphocytic lymphoma
Autoimmune cytopenia does not predict poor prognosis in chronic lymphocytic leukemia/small lymphocytic lymphoma
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DOI：
10.1002/ajh.10369
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发表时间：
2003-09-01
影响因子：
12.8
通讯作者：
Zent, CS
中科院分区：
文献类型：
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作者：
Kyasa, MJ;Parrish, RS;Zent, CS
慢性淋巴细胞性白血病/小淋巴细胞性淋巴瘤（CLL/SLL）的特征在于可引起自身免疫并发症（包括贫血和血小板减少症）的获得性免疫缺陷。我们在来自大人群（> 45，000名退伍军人）的132名患者中进行了CLL/SLL自身免疫并发症的流行病学，临床表现和意义的观察性研究，其中至少90%的CLL/SLL患者先前已被确定。在12.5年的时间内，12例患者（9.1%）有自身免疫性并发症;其中6例（4.5%）有自身免疫性溶血性贫血（AIHA），5例（3.8%）有免疫性血小板减少症（ITP），1例（0.8%）有纯红细胞再生障碍性贫血（PRBA）。6例AIHA直接免疫球蛋白试验IgG、C3 d均阳性。在6例患者中，CLL/SLL是在出现自身免疫性血细胞减少症时的偶然发现。10例患者中有9例对免疫抑制治疗有反应，7例患者并发严重感染，其中1例死亡。CLL/SLL自身免疫并发症患者的主要死亡原因是继发性恶性肿瘤。免疫性血细胞减少患者的生存率与无免疫性血细胞减少的CLL/SLL患者无显著差异。在贫血或血小板减少症患者中，与自身免疫性病因相比，骨髓衰竭患者的死亡率明显较高。我们发现，在CLL/SLL发病率高的非转诊人群中，自身免疫性血细胞减少症可发生在疾病自然史的早期。这些数据表明，Rai和Binet分类CLL需要修改自身免疫性血细胞减少症患者。2003年出版Wiley-Liss，Inc.（匕首）。
Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is characterized by an acquired immune defect that can cause autoimmune complications, including anemia and thrombocytopenia. We conducted an observational study of the epidemiology, clinical presentation and significance of autoimmune complications of CLL/SLL in 132 patients from a large population (>45,000 veterans), in which at least 90% of patients with CLL/SLL have been previously identified. Over a period of 12.5 years, 12 patients (9.1%) had autoimmune complications; of these, 6 (4.5%) had autoimmune hemolytic anemia (AIHA), 5 (3.8%) had immune thrombocytopenia (ITP), and 1 (0.8%) had pure red blood cell aplasia (PRBA). All 6 cases of AIHA had a positive direct immunoglobulin test for IgG and C3d. In 6 patients, CLL/SLL was an incidental finding at the time of presentation with autoimmune cytopenia. Nine out of 10 patients responded to immunosuppressive therapy, which was complicated by serious infection in 7 cases, one of which was fatal. The major cause of mortality in patients with autoimmune complications of CLL/SLL was secondary malignancy. Survival of patients with immune cytopenia was not significantly different from CLL/SLL patients without immune cytopenia. Among patients with anemia or thrombocytopenia, mortality was significantly higher in those with bone marrow failure compared to an autoimmune etiology. We show that in a non-referred population with a high incidence of CLL/SLL, autoimmune cytopenia can occur early in the natural history of the disease. These data suggest that the Rai and Binet classifications for CLL need to be modified for patients with autoimmune cytopenia. Published 2003 Wiley-Liss, Inc.(dagger).