淋巴瘤所致冷凝集素综合征3例临床回顾性分析并文献复习  

Clinical Retrospective Analysis of 3 Cases with Cold Agglutinin Syndrome in Lymphoma and Literature Review

作　　者：余锋 孙令凤 王娟 沈美晓 雷美清[1] 曾照成 罗贤生[1] 陈晓霞[1] 王智明[1] 杨晓阳[1] YU Feng;SUN Lingfeng;WANG Juan;SHEN Meixiao;LEI Meiqing;ZENG Zhaocheng;LUO Xiansheng;CHEN Xiaoxia;WANG Zhiming;YANG Xiaoyang(Department of Hematology,Haikou Municipal Hospital/the Affiliated Haikou Hospital,Xiangya School of Medicine,Central South University,Hainan,Haikou,570208,China)

机构地区：[1]中南大学湘雅医学院附属海口医院/海口市人民医院血液科

出　　处：《肿瘤药学》2019年第4期699-704,共6页Anti-Tumor Pharmacy

摘　　要：目的回顾性分析单中心淋巴瘤所致冷凝集素综合征的临床表现、实验室检查特征及预后。方法收集2011年12月-2018年11月我院收治的3例淋巴瘤所致冷凝集素综合征患者的病历资料,分析其临床特征、实验室检查结果及生存情况。结果3例淋巴瘤所致冷凝集素综合征患者中包括2例女性,1例男性,中位年龄33(24~73)岁,2例以贫血症状急性加重就诊,1例以指端坏死就诊,3例患者均有明确诱发加重因素;中位血红蛋白52(49~88)g·L^-1,中位冷凝集素滴度1∶512(1∶64~1∶1024);2例患者继发于弥漫大B细胞淋巴瘤,1例继发于淋巴浆细胞淋巴瘤/华氏巨球蛋白血症,均弥漫侵犯骨髓,免疫表型均表达CD20+B细胞淋巴瘤,轻链限制性表达。2例患者行R-CHOP方案化疗6个疗程,其中1例化疗2个疗程后评估达PR,4个疗程后病情进展,6个疗程后死亡,生存时间6个月;1例化疗1个疗程后评估达PR,2个疗程后达CR,生存时间23个月(存活至今)。另1例患者无治疗意愿,仅坏死指端局部保暖治疗,局部症状有所改善,生存时间16个月(存活至今)。结论冷凝集素综合征具有对冷接触相关的特殊临床表现,寒冷、感染、创伤、手术等均可诱发加重溶血,应尽量避免诱发加重因素并积极治疗,其长期疗效和预后取决于其原发疾病的治疗。Objective To retrospectively analyze the clinical manifestation, features of laboratorial examination results and prognosis of patients with lymphoma-caused cold agglutinin syndrome. Methods The clinical data of three cases of cold agglutinin syndrome in lymphoma admitted into our hospital between December 2011 and November 2018 were collected, and their clinical features, laboratorial examination results and long-term survival were analyzed. Results Among the three cases of cold agglutinin syndrome in lymphoma, two were female and one was male. The median age of them was 33(24-73)years. Two cases received diagnosis for acute exacerbation of symptoms of anemia. One case went to hospital for fingertip necrosis. All the three cases had definite aggravating factors. Median hemoglobin was 52(49-88) g·L-1. The titer of the median cold agglutinin was 1∶512(1:64-1∶1024). Two patients were secondary to diffuse large B-cell lymphoma, and the other to Waldenstr?m Macroglobulinemia/Lymphoplasmacytic. All of them got diffused invasion of bone marrow. CD20+ B cell lymphoma was expressed in all immunophenotypes with restrictive expression of light chain. Two cases received six courses of R-CHOP chemotherapy. One of the two was assessed in partial remission(PR) after two courses of treatment, in progress after four courses, and died after six courses. The survival time was six months. The other was assessed in PR after one course of chemotherapy, and complete remission(CR) after two courses. The survival time was 23 months(still alive). One case had no intention of treatment and was willing to only get local thermal treatment of fingertip necrosis. Local symptoms improved, and the patient survived 16 months(still alive). Conclusion Cold agglutinin syndrome has specific clinical manifestations associated with cold contact. Cold, infection, trauma, surgery and so on can induce acute phase hemolysis. Patients should try to avoid from the aggravating factors and get actively treatment. The long-term efficacy and prognosis of c

关 键 词：冷凝集素综合征 淋巴瘤 自身免疫性溶血性贫血 冷凝集素 

分 类 号：R733.4[医药卫生—肿瘤] R730.6[医药卫生—临床医学]