ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2021, Vol. 30 ›› Issue (3): 294-298.DOI: 10.3969/j.issn.1006298X.2021.03.021

• • 上一篇    

达雷妥尤单抗治疗复发难治性系统性轻链型淀粉样变性

  

  • 出版日期:2021-06-28 发布日期:2021-06-22

Daratumumab in a patient with relapsed light chain amyloidosis

  • Online:2021-06-28 Published:2021-06-22

摘要: 48岁女性患者,确诊为系统性κ轻链型淀粉样变性(累及肾脏、心脏、皮肤),骨髓浆细胞25%,荧光原位杂交(FISH)示1q21扩增及t(11;14),经硼替佐米+地塞米松方案化疗和大剂量美法仑联合自体造血干细胞移植治疗后反复复发,血液学进展,肾脏治疗无反应,故而改为达雷妥尤单抗+地塞米松(DD)方案。DD方案治疗1次后即达血液学非常好的部分缓解,治疗4次血液学完全缓解(CR)。随访3个月,血液学持续CR,肾脏无反应。


关键词: 轻链型淀粉样变性, 复发, 达雷妥尤单抗

Abstract: 48yearold woman was diagnosed as kappa light chain amyloidosis with kidney and heart involvement.Bone marrow biopsy revealed 25% plasma cells and FISH analysis showed gain of 1q21 and translocation t(11;14).A combination of bortezomib and dexamethasone was initiated but after 7 cycles,she developed progressive disease and therapy was switched to highdose melphalan and autologous stem cell transplantation (ASCT).She achieved a very good partial response (VGPR) at day 30.However her serum kappa light chain increased 4 months after ASCT and treatment with BD was restarted.Her best hematologic response was PR as ever,and lasted for only 5 months.Then she was treated with daratumumab and dexamethasone weekly for 4 weeks.She achieved VGPR after the 1st week and complete remission (CR) after the 4th weeks.After an interval of 1 month she proceed with another 4 doses of daratumumab every other week and remained hematologic CR during the followup,without renal response.

Key words: light chain amyloidosis, relapsed, daratumumab