Yasuda Yohei, Maki Hiroaki, Shimura Arika, Honda Akira, Masamoto Yosuke, Kurokawa Mineo
Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
Department of Cell Therapy and Transplantation Medicine, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
Int J Hematol. 2025 Mar;121(3):411-415. doi: 10.1007/s12185-024-03911-z. Epub 2025 Feb 4.
Type I cryoglobulinemia is typically associated with hematological malignancies such as B-cell lymphomas and plasma cell neoplasms. Its treatment basically targets underlying hematological malignancies, and the prognosis remains unsatisfactory. Despite several reports of type I cryoglobulinemia treated with bortezomib-based regimens, little is available on the treatment of bortezomib-resistant cases. We report a case of severe type I cryoglobulinemia associated with plasma cell neoplasm, refractory to bortezomib and daratumumab, which was successfully managed with a combination of carfilzomib, cyclophosphamide, and dexamethasone (KCd therapy). No sign of relapse has been seen for more than 3 years with maintenance therapy with ongoing carfilzomib. This case highlights the potential efficacy of carfilzomib-based regimens in bortezomib-resistant type I cryoglobulinemia, offering a promising option for cases refractory to conventional treatments.
I型冷球蛋白血症通常与血液系统恶性肿瘤相关，如B细胞淋巴瘤和浆细胞肿瘤。其治疗主要针对潜在的血液系统恶性肿瘤，预后仍不尽人意。尽管有几篇关于用硼替佐米方案治疗I型冷球蛋白血症的报道，但关于硼替佐米耐药病例的治疗资料很少。我们报告一例与浆细胞肿瘤相关的严重I型冷球蛋白血症病例，对硼替佐米和达雷妥尤单抗耐药，通过卡非佐米、环磷酰胺和地塞米松联合治疗（KCd疗法）成功治愈。持续使用卡非佐米维持治疗3年多未见复发迹象。该病例突出了基于卡非佐米的方案在硼替佐米耐药的I型冷球蛋白血症中的潜在疗效，为传统治疗难治的病例提供了一个有前景的选择。
