孙秀娟, 陈佳雯, 王婷玉, 王齐, 阎禹廷, 熊文婕, 邱录贵, 易树华. 14例慢性淋巴细胞白血病合并自身免疫性溶血性贫血患者的临床特征及预后分析[J]. 中国肿瘤临床, 2022, 49(20): 1062-1066. DOI: 10.12354/j.issn.1000-8179.2022.20220076
引用本文: 孙秀娟, 陈佳雯, 王婷玉, 王齐, 阎禹廷, 熊文婕, 邱录贵, 易树华. 14例慢性淋巴细胞白血病合并自身免疫性溶血性贫血患者的临床特征及预后分析[J]. 中国肿瘤临床, 2022, 49(20): 1062-1066. DOI: 10.12354/j.issn.1000-8179.2022.20220076
Xiujuan Sun, Jiawen Chen, Tingyu Wang, Qi Wang, Yuting Yan, Wenjie Xiong, Lugui Qiu, Shuhua Yi. Clinical features and prognosis of 14 patients with chronic lymphocytic leukemia complicated with autoimmune hemolytic anemia[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2022, 49(20): 1062-1066. DOI: 10.12354/j.issn.1000-8179.2022.20220076
Citation: Xiujuan Sun, Jiawen Chen, Tingyu Wang, Qi Wang, Yuting Yan, Wenjie Xiong, Lugui Qiu, Shuhua Yi. Clinical features and prognosis of 14 patients with chronic lymphocytic leukemia complicated with autoimmune hemolytic anemia[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2022, 49(20): 1062-1066. DOI: 10.12354/j.issn.1000-8179.2022.20220076

14例慢性淋巴细胞白血病合并自身免疫性溶血性贫血患者的临床特征及预后分析

Clinical features and prognosis of 14 patients with chronic lymphocytic leukemia complicated with autoimmune hemolytic anemia

  • 摘要:
      目的  探讨慢性淋巴细胞白血病(chronic lymphocytic leukemia,CLL)合并自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)的临床特征、治疗方案及疗效。
      方法  回顾性分析1989年1月至2013年12月中国医学科学院血液病医院确诊的14例CLL合并AIHA患者的临床资料。
      结果  14例CLL合并AIHA的患者中,其中男性11例,女性3例,中位年龄57.5(42.0~69.0)岁。B症状4例(28.6%),贫血9例(64.3%),乳酸脱氢酶(lactate dehydrogenase,LDH)增高10例(71.4%)。针对AIHA进行治疗后,14例患者中可评价疗效者9例,β2-微球蛋白水平增高9例(100%),免疫球蛋白重链可变区(immunoglobulin heavy chain variable region,IGHV)无突变3例(37.5%)。总有效(overall response rate,ORR)率为100%(9/9),完全缓解(complete response,CR)为6 例(66.7%),部分缓解(partial response,PR)为3例(33.3%)。针对CLL进行治疗后,14 例患者中可评价疗效者11例,ORR为63.6%(7/11),CR为3例(27.3%),PR为4例(36.4%),疾病稳定(stable disease,SD)为3例(27.3%),疾病进展(progressive disease,PD)为1 例(9.1%)。有长期随访资料的11 例患者中,中位无进展生存期 (median progression-free survival,mPFS)为 69(4~120)个月,中位总生存期(median overall survival ,mOS)为 75(4~128)个月。5 年无进展生存(progression-free survival,PFS)率为(62.3±15.0)%,10年PFS率为(18.7±15.5)%,5年总生存(overall survival,OS)率为(80.0±12.6)%,10 年 OS 率为(24.0±19.5)%。
      结论  AIHA为CLL的常见并发症,可在疾病不同阶段出现。糖皮质激素及美罗华联合化疗疗效较好,新型靶向药物具有良好的应用前景。

     

    Abstract:
      Objective  To investigate clinical characteristics of and efficacy of treatments for chronic lymphocytic leukemia (CLL) complicated with autoimmune hemolytic anemia (AIHA).
      Methods  Clinical data of 14 patients with CLL complicated with AIHA were retrospectively analyzed in Institute of Hematology & Blood Diseases Hospital from January 1989 to December 2013.
      Results  The study included 14 patients comprising 11 males and 3 females, and the median age of the patients was 57.5 years (range 42–69 years). Before treatment, four patients (28.6%) had symptom B, 9 (64.3%) had anemia, 10 (71.4%) had high lactate dehydrogenase (LDH) levels. After treatment with AIHA, 9 of the 14 patients were evaluated, 9 (100%) had high β2-microglobulin levels, and 3 (37.5%) had unmutated immunoglobulin heavy chain variable region (IGHV). The overall response rate (ORR) was 100% (9/9), and 6 (66.7%) patients had complete response (CR) , 3 (33.3%) had partial response (PR) . After treatment for CLL, 11 of the 14 patients were evaluated. The ORR was 63.6% (7/11). Three patients had CR (27.3%), 4 had PR (36.4%), 3 had stable disease (SD) (27.3%), and 1 had progressive disease (PD) (9.1%). In 11 patients with long-term follow-up data, the median PFS was 69 (4–120) months, and the median OS was 75 (4-128) months. The 5- and 10-year PFS were (62.3±15.0)% and (18.7±15.5)%, respectively, and the 5- and 10-year OS were (80.0±12.6)% and (24.0±19.5)%, respectively.
      Conclusions  AIHA is a common complication of CLL and can occur at different stages of CLL. Treatment with corticosteroids and rituximab combined with chemotherapy had a therapeutic effect in these patients, and the new targeted drugs are effective for CLL complicated with AIHA.

     

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