单克隆免疫球蛋白血症相关纯红细胞再生障碍性贫血2例并文献复习  

Pure red cell aplasia associated with monoclonal gammopathy:two cases report and review of the literature

作　　者：孔令芳 宋姣杰 李欣然 韩欢 赵丽云[1] KONG Lingfang;SONG Jiaojie;LI Xinran;HAN Huan;ZHAO Liyun(Department of Hematology,Xingtai People's Hospital,Xingtai,054100,China)

机构地区：[1]邢台市人民医院血液科,河北邢台054100

出　　处：《临床血液学杂志》2024年第11期826-829,共4页Journal of Clinical Hematology

摘　　要：单克隆免疫球蛋白血症相关纯红细胞再生障碍性贫血国内外报道较为少见,文章报道了2例单克隆免疫球蛋白血症相关纯红细胞再生障碍性贫血患者的诊疗,并进行相关文献复习。2例患者初诊时均为重度贫血,伴有低水平M蛋白,骨髓中浆细胞比例低于10%,完善骨髓相关检查后诊断纯红细胞再生障碍性贫血、单克隆免疫球蛋白血症,给予环孢素、糖皮质激素等治疗后无明显好转,给予含硼替佐米的抗浆细胞治疗后,2例患者均在治疗1个疗程后取得明显疗效,脱离输血,其中1例患者在2个疗程治疗后M蛋白转阴,血红蛋白恢复正常,1例患者在1个疗程治疗后血红蛋白升至正常,2个疗程后M蛋白转阴,2例患者均在化疗3个疗程后停药,其中1例患者随访至今27个月,1例患者随访至今42个月,血红蛋白均在正常范围,M蛋白阴性。含硼替佐米抗浆细胞治疗方案对于单克隆免疫球蛋白血症相关纯红细胞再生障碍性贫血治疗有效,为单克隆免疫球蛋白血症相关纯红细胞再生障碍性贫血的治疗提供了新的方法。Reports on pure red cell aplasia associated with monoclonal gammopathy are relatively rare at home and abroad,This article reports two cases of pure red cell aplasia associated with monoclonal gammopathy and reviews related literature.Both cases were severe anemia with low levels of M protein at initial diagnosis.The proportion of plasma cells in bone marrow was less than 10%,After completing bone marrow related examination,pure red cell aplastic anemia and monoclonal gammopathy were diagnosed,and there were no significant improvements after treatment with cyclosporine and glucocorticoid.After anti-plasma cell treatment with bortezomib,both patients achieved significant curative effect after one course of treatment and were free from blood transfusion.Among them,M protein was undetectable in 1 patient after 2 courses of treatment,and the hemoglobin returned to normal range.The other patient's hemoglobin returned to the normal range after 1 course of treatment,and M protein was negative after 2 courses of treatment.Treatment was discontinued in both patients after 3 courses of chemotherapy.Among them,1 patient was followed up for 27 months and the other was followed up for 42 months,and hemoglobin levels remaining in the normal range,and M protein were undetectable.Antiplasma cell therapy regimen containing bortezomib is effective for the treatment of pure red cell aplasia associated with monoclonal gammopathy,and provides a new method for the treatment of pure red cell aplasia associated with monoclonal gammopathy.

关 键 词：纯红细胞再生障碍性贫血 单克隆免疫球蛋白血症 硼替佐米 

分 类 号：R556.5[医药卫生—血液循环系统疾病]