Proteasome inhibitors (PIs) are crucial in treating multiple myeloma but carry a risk of thrombotic microangiopathy (TMA), especially with carfilzomib use. This systematic review includes 44 studies with 115 cases of PI-induced TMA, where carfilzomib was implicated in 101 cases. Treatment approaches varied: 28 patients received supportive care, 43 underwent therapeutic plasma exchange (TPE), 9 were treated exclusively with eculizumab (ECU), and 13 received both TPE and ECU. Notably, eculizumab significantly improved outcomes for patients unresponsive to initial TPE, achieving complete remission in seven cases. The need for dialysis emerged as a significant predictor of outcomes, often indicating a poor prognosis. For patients suspected of having PI-TMA, it is advisable to discontinue the offending medication promptly, even without definitive laboratory confirmation. In cases where diagnosis is challenging, kidney biopsy may assist if conditions permit. Comprehensive evaluation of the complement system, including genetic mutations, function, and associated complement inhibitory factor antibodies, should be included in the assessment of PI-TMA. Early administration of eculizumab may be beneficial in cases of suspected complement abnormalities or suboptimal response to initial treatments.
蛋白酶体抑制剂在治疗多发性骨髓瘤中至关重要，但存在引发血栓性微血管病的风险，尤其是使用卡非佐米时。本系统综述纳入44项研究，共115例蛋白酶体抑制剂诱导的血栓性微血管病病例，其中101例与卡非佐米相关。治疗方案多样：28例患者接受支持治疗，43例行治疗性血浆置换，9例仅使用依库珠单抗治疗，13例联合接受血浆置换与依库珠单抗治疗。值得注意的是，对于初始血浆置换反应不佳的患者，依库珠单抗显著改善了临床结局，其中7例达到完全缓解。需要透析成为预后的重要预测因素，通常提示不良结局。对于疑似蛋白酶体抑制剂相关血栓性微血管病的患者，即使缺乏明确的实验室证据，也建议及时停用相关药物。在诊断困难的情况下，若条件允许可通过肾活检辅助诊断。对蛋白酶体抑制剂相关血栓性微血管病的评估应包括补体系统的全面检测，涵盖基因突变、功能及相关补体抑制因子抗体。对于疑似补体异常或初始治疗反应不佳的病例，早期应用依库珠单抗可能有益。
Proteasome inhibitors related thrombotic microangiopathy: a systematic and comprehensive review
肿瘤(癌症)患者之家