Kuan Jew Win, Chai Sing Ling, Rajadurai Pathmanathan, Lau Lee Gong, Uchang Joseph, Syed Husain Sharifah Noor Akmal
Department of Medicine, Universiti Malaysia Sarawak, Kota Samarahan, Sarawak, Malaysia.
Timberland Medical Centre, Kuching, Sarawak, Malaysia.
Case Rep Hematol. 2022 Aug 31;2022:4081971. doi: 10.1155/2022/4081971. eCollection 2022.
Central nervous system (CNS) involvement in multiple myeloma (MM) (MM-CNS) in the form of leptomeningeal myelomatosis or brain parenchyma plasmacytoma is rare, causing challenges in clinical diagnosis and treatment. We would like to report a case of leptomeningeal myelomatosis and illustrated the challeges. A 61-year-old man was diagnosed with MM with left paravertebral plasmacytoma, R-ISS II with high suspicion of double-hit MM, either biallelic aberrancy of TP53 or del(17p) and IGH aberrancy depending on the definition chosen, treated with lenalidomide-bortezomib-dexamethasone and local radiotherapy, later developed systemic relapse and progression to MM-CNS in the form of leptomeningeal myelomatosis. A modified CNS-based treatment not reported before, consisting of daratumumab, pomalidomide, vincristine, procarbazine, and dexamethasone, brought a rapid clinical improvement and warrants a further study. Incorporation of intrathecal thiotepa into the regimen would likely increase the efficacy.
中枢神经系统(CNS)以柔脑膜骨髓瘤病或脑实质浆细胞瘤的形式累及多发性骨髓瘤(MM)(MM-CNS)较为罕见,给临床诊断和治疗带来挑战。我们报告一例柔脑膜骨髓瘤病病例并阐述相关挑战。一名61岁男性被诊断为MM伴左椎旁浆细胞瘤,R-ISS II期,高度怀疑双打击MM,根据所选定义,存在TP53双等位基因异常或del(17p)以及IGH异常,接受来那度胺-硼替佐米-地塞米松治疗及局部放疗,随后出现全身复发并进展为柔脑膜骨髓瘤病形式的MM-CNS。一种此前未报道的基于CNS的改良治疗方案,由达雷妥尤单抗、泊马度胺、长春新碱、丙卡巴肼和地塞米松组成,带来了快速的临床改善,值得进一步研究。将鞘内注射噻替派纳入该方案可能会提高疗效。