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Effectiveness of complement inhibitors against refractory antibody-mediated rejection of lung transplantation: Two clinical cases.
Transpl Immunol
January 2025
Univ. Grenoble Alpes, CNRS, Pharmacy Department, TIMC, UMR5525, Grenoble Alpes University, Grenoble, France.
Antibody-mediated rejection (AMR) has been recognized as a significant cause of acute and chronic lung allograft dysfunction after lung transplantation. Some treatments, eculizumab, an anti-complement (C)5 component monoclonal antibody (Mab), seem to have a promising effect in the management of some patients with AMR. We present two patients with acute AMR after lung transplantation who received the anti-C5 Mab therapy.
View Article and Find Full Text PDFCatastrophic antiphospholipid syndrome: a CAPS-tivating hematologic disease.
Hematology Am Soc Hematol Educ Program
December 2024
Department of Medicine, McMaster University, Hamilton, Ontario, Canada.
Catastrophic antiphospholipid syndrome (CAPS) is a rare but life-threatening form of antiphospholipid syndrome (APS) defined by the rapid onset of large and small vessel thrombosis occurring simultaneously across multiple sites, resulting in multiorgan dysfunction. The presence of underlying immune dysfunction causing activation of coagulation and, in many cases, abnormal complement regulation predisposes these patients to thrombotic events. CAPS is often preceded by triggering factors such as infection, surgery, trauma, anticoagulation discontinuation, and malignancy.
View Article and Find Full Text PDFTreatment discontinuation in adults with atypical hemolytic uremic syndrome (aHUS): a qualitative study of international experts' perspectives with associated cost-consequence analysis.
BMC Nephrol
November 2024
Division of Hematology and Medical Oncology, Weill Cornell Medical College, 1300 York Avenue, New York, NY, 10065, USA.
Background: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA) related to congenital mutations impeding control of the alternative pathway of complement. Following approval of the complement C5 inhibitor eculizumab by the European Medicines Agency and the US Food and Drug Administration, initial guidelines suggested lifelong therapy. Yet, growing evidence indicates that discontinuation of eculizumab, or its long-acting form ravulizumab, is possible for many patients.
View Article and Find Full Text PDFAcute Myeloid Leukemia as a Trigger for Hemolytic-Uremic Syndrome.
J Clin Med
October 2024
Department of Nephrology, Fundació Puigvert, 08025 Barcelona, Spain.
Article Synopsis
- Acute myeloid leukemia (AML) is usually not linked to secondary hemolytic-uremic syndrome (HUS), but this case report presents an exception.
- A woman diagnosed with AML developed severe HUS, and her condition improved significantly after receiving eculizumab, a medication that helped stabilize her health.
- After one year with normal renal function and genetic tests, she was able to stop eculizumab, underscoring the importance of early treatment for secondary HUS in improving patient outcomes.
Proteasome inhibitors related thrombotic microangiopathy: a systematic and comprehensive review.
Blood Cancer J
November 2024
Department of Hematology, Affiliated Hangzhou First People's Hospital, Westlake University, School of Medicine, Hangzhou, China.
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.
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