Wu Y Y, Cao X X, Cai H, Zhang L, Zhou D B, Li J
Department of Hematology, Peking Union Medical College Hospital, CAMS & PUMC, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2017 Sep 14;38(9):789-793. doi: 10.3760/cma.j.issn.0253-2727.2017.09.011.
To explore the clinical characteristics, treatment and prognosis in 17 patients with primary cold agglutinin disease (CAD) . Clinical data, treatment and survival status of 17 patients diagnosed with primary cold agglutinin disease in Peking Union Medical College Hospital during April 2007 to October 2016 were retrospectively analyzed. The MYD88(L265P) mutation was tested in 4 patients. The median age of 17 patients was 67 years (range, 51-86 years) , and male- to female ratio was 1.1∶1. Seven patients were diagnosed with indolent lymphoma, including 3 Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) , 2 small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL) , and 2 splenic marginal zone lymphoma (SMZL) . 15 patients experienced anemia. The median HGB level was 67 (35-127) g/L. 11 patients had cold agglutinin (CA) titers ≥1∶64, with median CA of 1∶1 024. MYD88(L265P) mutation was detected in 1 patient. 12 patients received drug therapy: 7 were treated with glucocorticoid-based therapy and 1 patient responded to treatment; 5 received rituximab-based therapy and 3 patients responded to treatment. With a median follow-up of 14 (0.5-96) months, the median overall survival was not reached. Clinical manifestations of CAD are various, and diagnosis is dependent on CA testing. The efficacy of glucocorticoid-based therapy is limited, and rituximab is recommended for CAD treatment.
探讨17例原发性冷凝集素病（CAD）患者的临床特征、治疗及预后。回顾性分析2007年4月至2016年10月在北京协和医院确诊为原发性冷凝集素病的17例患者的临床资料、治疗及生存状况。对4例患者检测了MYD88（L265P）突变。17例患者的中位年龄为67岁（范围51 - 86岁），男女比例为1.1∶1。7例患者被诊断为惰性淋巴瘤，其中3例为华氏巨球蛋白血症/淋巴浆细胞淋巴瘤（WM/LPL），2例为小淋巴细胞淋巴瘤/慢性淋巴细胞白血病（SLL/CLL），2例为脾边缘区淋巴瘤（SMZL）。15例患者出现贫血。血红蛋白（HGB）水平中位数为67（35 - 127）g/L。11例患者冷凝集素（CA）滴度≥1∶64，CA中位数为1∶1024。1例患者检测到MYD88（L265P）突变。12例患者接受了药物治疗：7例接受以糖皮质激素为主的治疗，1例治疗有反应；5例接受以利妥昔单抗为主的治疗，3例治疗有反应。中位随访时间为14（0.5 - 96）个月，总生存中位数未达到。CAD的临床表现多样，诊断依赖于CA检测。以糖皮质激素为主的治疗疗效有限，推荐利妥昔单抗用于CAD治疗。
