Good treatment-free survival of monoclonal gammopathy of undetermined significance associated pure red cell aplasia after bortezomib plus dexamethasone

被引:4
作者
Zhang, Lele [1 ]
Chen, Nafei [2 ]
Xu, Zhipeng [3 ]
Liang, Qian [1 ]
Pan, Hong [1 ]
Zhao, Jingyu [1 ]
Fang, Liwei [1 ]
Shi, Jun [1 ]
机构
[1] Chinese Acad Med Sci & Peking Union Med Coll, Regenerat Med Clin, State Key Lab Expt Hematol, Natl Clin Res Ctr Blood Dis,Inst Hematol & Blood, Tianjin 300020, Peoples R China
[2] Hebei Med Univ Affiliated Hosp, Xingtai Peoples Hosp, Shijiazhuang 054000, Hebei, Peoples R China
[3] Shanxi Datong Univ, Peoples Hosp 2, Datong Canc Hosp, Affiliated Canc Hosp, Datong 037005, Shanxi, Peoples R China
关键词
Bortezomib; Pure red cell aplasia; Monoclonal gammopathy of undetermined significance; Treatment free survival; MULTIPLE-MYELOMA; MANAGEMENT;
D O I
10.1016/j.bcmd.2021.102573
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Pure red cell aplasia (PRCA) is a rare syndrome characterized by severe anemia and absence of erythroid precursors. PRCA associated to monoclonal gammopathy of undetermined significance (MGUS) is a scarce condition with less than five cases reported so far. There is no agreement on the treatment of MGUS associated PRCA and treatment- free survival (TFS) is an unmet clinical need. In this report, for the first time, we demonstrated two patients with MGUS associated PRCA obtained rapid remission and maintained TFS after accepting intensive short-term bortezomib plus dexamethasone. The first case was refractory to cyclosporine and prednisone, but achieved complete remission after ten doses of bortezomib. Moreover, he has kept TFS for 12 months. The other case initiated bortezomib plus dexamethasone as soon as making a definite diagnosis. She obtained complete remission after twelve doses of bortezomib and she has maintained a normal level of haemoglobin for 8 months.
引用
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页数:4
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