Röth Alexander, Kulasekararaj Austin G, Scheinberg Phillip, Nishimura Jun-Ichi
Department of Hematology and Stem Cell Transplantation, West German Cancer Center, University Hospital Essen, University of Duisburg-Essen, Essen, Germany.
Department of Hematological Medicine, King's College Hospital, London, UK.
Immunotherapy. 2024;16(20-22):1185-1196. doi: 10.1080/1750743X.2024.2433410. Epub 2024 Dec 2.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired, rare, life-threatening hematopoietic stem cell disorder that causes stem cell-derived cells to be vulnerable to complement-mediated lysis and manifests as hemolytic anemia, thrombosis, and peripheral blood cytopenias. C5 inhibitors, eculizumab, and ravulizumab, are recognized as the current standard of care for PNH treatment in countries where they are available. Crovalimab (PiaSky®), which is approved for the treatment of PNH, is a novel anti-C5 inhibitor with an every-4-weeks, low-volume, subcutaneous maintenance dosing regimen with the possibility for self-administration. Data from three phase III studies highlight the overall favorable benefit-risk profile of crovalimab, showing that crovalimab has promising potential to address the unmet medical and socioeconomic challenges in the PNH treatment landscape.
阵发性睡眠性血红蛋白尿症(PNH)是一种后天性、罕见且危及生命的造血干细胞疾病,它会导致干细胞衍生的细胞易受补体介导的溶解作用影响,并表现为溶血性贫血、血栓形成和外周血细胞减少。在有C5抑制剂(依库珠单抗和ravulizumab)可用的国家,它们被公认为是PNH治疗的当前标准疗法。已获批用于治疗PNH的crovalimab(PiaSky®)是一种新型抗C5抑制剂,采用每4周一次的小剂量皮下维持给药方案,且患者有可能自行给药。三项III期研究的数据突出了crovalimab总体良好的获益风险特征,表明crovalimab在应对PNH治疗领域尚未满足的医学和社会经济挑战方面具有广阔的潜力。