Complement blockade with a C1 esterase inhibitor in paroxysmal nocturnal hemoglobinuria
نویسندگان
چکیده
منابع مشابه
Intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.Acute abdominal pain is one of the PNH clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.Eculizumaband allogeneic bone marrow transplantation (BMT) arethe only w...
متن کاملEffect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria.
Hemolysis and hemoglobinemia contribute to serious clinical sequelae in hemolytic disorders. In paroxysmal nocturnal hemoglobinuria (PNH) patients, hemolysis can contribute to thromboembolism (TE), the most feared complication in PNH, and the leading cause of disease-related deaths. We evaluated whether long-term treatment with the complement inhibitor eculizumab reduces the rate of TE in patie...
متن کاملTRANSFUSION MEDICINE Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria
1Leeds General Infirmary, Leeds, United Kingdom; 2Radboud University Medical Center, Nijmegen, The Netherlands; 3University Essen, Essen, Germany; 4Federico II University, Naples, Italy; 5Saarland University Medical School, Homburg-Saarland, Germany; 6Istituto Toscano Tumori, Florence, Italy; 7Institute of Transfusion Medicine, University Hospital, Ulm, Germany; 8Royal Melbourne Hospital, Melbo...
متن کاملThe use of the complement inhibitor eculizumab (Soliris®) for treating Korean patients with paroxysmal nocturnal hemoglobinuria
BACKGROUND Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder characterized by chronic complement-mediated hemolysis. Eculizumab, a humanized monoclonal antibody against the terminal complement protein C5, potently reduces chronic intravascular hemolysis. We tested the clinical efficacy and safety of a 24-week treatment with eculizumab in 6 Korean patients with PNH. METH...
متن کاملParoxysmal nocturnal hemoglobinuria.
Paroxysmal Nocturnal Hemoglobinuria (PNH) literally means to have episodes of hemoglobin in the urine during the night. It is a Coomb's negative rare hemolytic disorder characterized by non-malignant clonal expansion of haemopoietic stem cells due to acquired genetic mutations. A 30 years old male patient presented with 5 years history of transfusion dependent anemia with intermittent episodes ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Experimental Hematology
سال: 2014
ISSN: 0301-472X
DOI: 10.1016/j.exphem.2014.06.007