Successful treatment with bortezomib in type-1 cryoglobulinemic vasculitis patient after rituximab failure: a case report and literature review
Successful treatment with bortezomib in type-1 cryoglobulinemic vasculitis patient after rituximab failure: a case report and literature review
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硼替佐米成功治疗利妥昔单抗失败后的 1 型冷球蛋白血症性血管炎患者：病例报告和文献综述
DOI：
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发表时间：
2013
影响因子：
2.1
通讯作者：
J. Nossent
中科院分区：
文献类型：
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作者：
E. Besada;A. Vik;W. Koldingsnes;J. Nossent
1型冷球蛋白血症性血管炎（CV）和混合型CV在病理生理学、临床表现和治疗反应方面存在差异。我们报告一位1型冷球蛋白血症性血管炎及皮肤溃疡的病人，尽管接受包括利妥昔单抗在内的多种免疫调节药物治疗，但仍保持活跃。该患者在CV发作后10年有非霍奇金淋巴瘤既往病史，该患者完全缓解。该患者随后出现血清和骨髓中的血液学异常，但未明确诊断为骨髓瘤。患者在硼替佐米和地塞米松治疗后最终进入临床缓解期。但她没有达到免疫缓解，仍然有阳性的冷球蛋白血症和血清κ型游离轻链。这表明硼替佐米，一种蛋白酶体抑制剂，抑制血管生成和副蛋白的产生，是一种有前途的治疗1型冷球蛋白血症血管炎。
Type-1 cryoglobulinemic vasculitis (CV) and mixed CV differ in their pathophysiology, clinical expression and treatment response. We report one patient with type-1 cryoglobulinemic vasculitis and skin ulcers that had remained active despite treatment with a variety of immunomodulating drugs including rituximab. The patient had a past medical history of non-Hodgkin lymphoma 10 years after CV onset for which she went into complete remission. The patient developed subsequent hematological anomalies in the serum and in the bone marrow without a definite diagnosis of myeloma. The patient finally went into clinical remission of her cryoglobulinemic vasculitis after treatment with bortezomib and dexamethasone. But she did not achieve an immunological remission and still had positive cryoglobulinemia and serum kappa-type free light chains. This suggests that bortezomib, a proteasome inhibitor that inhibits angiogenesis and production of paraproteins, is a promising treatment in type-1 cryoglobulinemic vasculitis.