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http://dx.doi.org/10.1016/j.nefro.2015.07.007 | DOI Listing |
Rinsho Ketsueki
September 2025
Department of Hematology and Oncology, Osaka University Graduate School of Medicine.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disorder characterized by complement-mediated hemolysis, thrombosis, and bone marrow failure. Eculizumab (Ecu), a C5 inhibitor, blocks intravascular hemolysis (IVH) and improves prognosis. Ravulizumab and crovalimab have longer half-lives, and reduce treatment burden.
View Article and Find Full Text PDFCureus
August 2025
Nephrology Department, Unidade Local de Saúde São João, Porto, PRT.
Carfilzomib is a second-generation proteasome inhibitor used in relapsed or refractory multiple myeloma (MM). Although effective, it can be associated with rare but life-threatening complications. We describe a 73-year-old woman with relapsed IgG-kappa MM who developed thrombotic microangiopathy (TMA) and acute kidney injury (AKI) requiring hemodialysis, three weeks after initiating a carfilzomib-based regimen.
View Article and Find Full Text PDFClin Exp Med
August 2025
Hematology Department, Kadhimiya Hematology Center, Baghdad, Iraq.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematological disorder caused by uncontrolled terminal complement activation of blood cells. It is associated with intravascular hemolysis, thromboembolic events, organ damage, impaired quality of life and premature mortality. As there are no PNH registry data from Middle Eastern countries, little is known about its management in the region.
View Article and Find Full Text PDFClin Transplant
September 2025
Department of Pediatrics and Medical Genetics, Ghent University Hospital, Ghent, Belgium.
Introduction: Atypical hemolytic uremic syndrome (aHUS) is a disease of complement dysregulation that may lead to kidney failure. Ravulizumab and eculizumab are complement C5 inhibitors approved for the treatment of aHUS. This study assessed the real-world effectiveness and safety of switching to ravulizumab from eculizumab in kidney transplant recipients with aHUS.
View Article and Find Full Text PDFAm J Hematol
August 2025
Department of Oncology and Hematology, University of Milan and Azienda Socio-Sanitaria Territoriale Papa Giovanni XXIII,Piazza OMS, Bergamo, Italy.
Inappropriate complement activation is a key driver of hematopoietic cell transplant-associated thrombotic microangiopathy (TA-TMA). Treatment with narsoplimab, an inhibitor of MASP-2, the effector enzyme of the lectin pathway, resulted in a response rate of 61% in a Phase 2 clinical trial in adults with TA-TMA. Given these promising results, a global expanded access program (EAP) was established facilitating compassionate use treatment.
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